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| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{Lymphoplasmacytic lymphoma}} |
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| {{CMG}}; {{AE}} {{S.M.}} | | {{CMG}}; {{AE}} {{S.M.}} |
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| {{SK}} Waldenstrom's macroglobulinemia; Waldenstrom's disease; Primary macroglobulinemia; Hyperviscosity syndrome | | {{SK}} Plasmacytoid lymphocytic [[lymphoma]]; Familial Waldenström's macroglobulinemia; Primary macroglobulinemia; Hyperviscosity syndrome; Lymphoplasmacytoid [[lymphoma]] |
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| ==Overview== | | ==[[Lymphoplasmacytic lymphoma overview|Overview]]== |
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| ==Historical Perspective== | | ==[[Lymphoplasmacytic lymphoma historical perspective|Historical Perspective]]== |
| *In 1944, [[Jan G. Waldenstrom]], a Swedish doctor of internal medicine, first discovered Waldenstrom macroglobulinemia(WM). He reported an unusal presentation of fatigue, lymphadenopathy, bleeding from nose and mouth, worsening anemia, elevated sedimentation rate, low serum fibrinogen levels (hypofibrinogenemia), hyperviscosity, and hypergammaglobulinemia in two patients due to increased levels of a class of an abnormal high molecular weight serum protein called macroglobulins.<ref>{{Cite journal
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| | author = [[Hatem Kaseb]] & [[Prerna Mewawalla]]
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| | title = Cancer, Lymphoma, Lymphoplasmacytic (Waldenstrom Macroglobulinemia)
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| | year = 2018
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| | month = January
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| | pmi
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| }}</ref><ref name="KonoplevMedeiros2005">{{cite journal|last1=Konoplev|first1=Sergej|last2=Medeiros|first2=L. Jeffrey|last3=Bueso-Ramos|first3=Carlos E.|last4=Jorgensen|first4=Jeffrey L.|last5=Lin|first5=Pei|title=Immunophenotypic Profile of Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia|journal=American Journal of Clinical Pathology|volume=124|issue=3|year=2005|pages=414–420|issn=0002-9173|doi=10.1309/3G1XDX0DVHBNVKB4}}</ref>
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| *In 1962, the first report on familiality in WM was published, and since then many cohort studies as well as small case-control studies have been published showing familial aggregation of WM.<ref name="pmid13933388">{{cite journal| author=MASSARI R, FINE JM, METAIS R| title=Waldenstrom's macroglobulinaemia observed in two brothers. | journal=Nature | year= 1962 | volume= 196 | issue= | pages= 176-8 | pmid=13933388 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13933388 }} </ref><ref name="pmid16224483">{{cite journal| author=Altieri A, Bermejo JL, Hemminki K| title=Familial aggregation of lymphoplasmacytic lymphoma with non-Hodgkin lymphoma and other neoplasms. | journal=Leukemia | year= 2005 | volume= 19 | issue= 12 | pages= 2342-3 | pmid=16224483 | doi=10.1038/sj.leu.2403991 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16224483 }} </ref><ref name="pmid6778280">{{cite journal| author=Blattner WA, Garber JE, Mann DL, McKeen EA, Henson R, McGuire DB et al.| title=Waldenström's macroglobulinemia and autoimmune disease in a family. | journal=Ann Intern Med | year= 1980 | volume= 93 | issue= 6 | pages= 830-2 | pmid=6778280 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6778280 }} </ref><ref name="pmid6805257">{{cite journal| author=Fine JM, Lambin P, Massari M, Leroux P| title=Malignant evolution of asymptomatic monoclonal IgM after seven and fifteen years in two siblings of a patient with Waldenström's macroglobulinemia. | journal=Acta Med Scand | year= 1982 | volume= 211 | issue= 3 | pages= 237-9 | pmid=6805257 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6805257 }} </ref><ref name="pmid3099545">{{cite journal| author=Fine JM, Muller JY, Rochu D, Marneux M, Gorin NC, Fine A et al.| title=Waldenström's macroglobulinemia in monozygotic twins. | journal=Acta Med Scand | year= 1986 | volume= 220 | issue= 4 | pages= 369-73 | pmid=3099545 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3099545 }} </ref><ref name="pmid408931">{{cite journal| author=Gétaz EP, Staples WG| title=Familial Waldenström's macroglobulinaemia: a case report. | journal=S Afr Med J | year= 1977 | volume= 51 | issue= 24 | pages= 891-2 | pmid=408931 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=408931 }} </ref><ref name="pmid8371587">{{cite journal| author=Linet MS, Humphrey RL, Mehl ES, Brown LM, Pottern LM, Bias WB et al.| title=A case-control and family study of Waldenstrom's macroglobulinemia. | journal=Leukemia | year= 1993 | volume= 7 | issue= 9 | pages= 1363-9 | pmid=8371587 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8371587 }} </ref><ref name="pmid8047841">{{cite journal| author=Ogmundsdóttir HM, Jóhannesson GM, Sveinsdóttir S, Einarsdóttir S, Hegeman A, Jensson O et al.| title=Familial macroglobulinaemia: hyperactive B-cells but normal natural killer function. | journal=Scand J Immunol | year= 1994 | volume= 40 | issue= 2 | pages= 195-200 | pmid=8047841 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8047841 }} </ref><ref name="pmid4143650">{{cite journal| author=Seligmann M, Danon F, Mihaesco C, Fudenberg HH| title=Immunoglobulin abnormalities in families of patients with Waldenström's macroglobulinemia. | journal=Am J Med | year= 1967 | volume= 43 | issue= 1 | pages= 66-83 | pmid=4143650 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4143650 }} </ref> <ref name="pmid1958390">{{cite journal| author=Taleb N, Tohme A, Abi Jirgiss D, Kattan J, Salloum E| title=Familial macroglobulinemia in a Lebanese family with two sisters presenting Waldenström's disease. | journal=Acta Oncol | year= 1991 | volume= 30 | issue= 6 | pages= 703-5 | pmid=1958390 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1958390 }} </ref><ref name="pmid16357024">{{cite journal| author=Treon SP, Hunter ZR, Aggarwal A, Ewen EP, Masota S, Lee C et al.| title=Characterization of familial Waldenstrom's macroglobulinemia. | journal=Ann Oncol | year= 2006 | volume= 17 | issue= 3 | pages= 488-94 | pmid=16357024 | doi=10.1093/annonc/mdj111 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16357024 }} </ref><ref name="pmid104746">{{cite journal| author=Youinou P, le Goff P, Saleun JP, Rivat L, Morin JF, Fauchier C et al.| title=Familial occurrence of monoclonal gammapathies. | journal=Biomedicine | year= 1978 | volume= 28 | issue= 4 | pages= 226-32 | pmid=104746 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=104746 }} </ref><ref name="pmid2505923">{{cite journal| author=Renier G, Ifrah N, Chevailler A, Saint-Andre JP, Boasson M, Hurez D| title=Four brothers with Waldenstrom's macroglobulinemia. | journal=Cancer | year= 1989 | volume= 64 | issue= 7 | pages= 1554-9 | pmid=2505923 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2505923 }} </ref>
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| *In 1994, a Revised European-American classification of lymphoid neaoplasms (REAL) was published by International Lymphoma Study Group which placed WM in the category of lymphoplasmacytic lymphoma (an indolent subtype of non-hodgkins lymphoma). The REAL classification is based on the morphology, immunophenotype, genetic features, and clinical features.<ref name="pmid10577857">{{cite journal| author=Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J et al.| title=World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. | journal=J Clin Oncol | year= 1999 | volume= 17 | issue= 12 | pages= 3835-49 | pmid=10577857 | doi=10.1200/JCO.1999.17.12.3835 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10577857 }} </ref><ref name="pmid8068936">{{cite journal| author=Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML et al.| title=A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. | journal=Blood | year= 1994 | volume= 84 | issue= 5 | pages= 1361-92 | pmid=8068936 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8068936 }} </ref>
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| *In 2001, WHO also classified the pathology of WM as lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia based on REAL classification.<ref name="KonoplevMedeiros2005">{{cite journal|last1=Konoplev|first1=Sergej|last2=Medeiros|first2=L. Jeffrey|last3=Bueso-Ramos|first3=Carlos E.|last4=Jorgensen|first4=Jeffrey L.|last5=Lin|first5=Pei|title=Immunophenotypic Profile of Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia|journal=American Journal of Clinical Pathology|volume=124|issue=3|year=2005|pages=414–420|issn=0002-9173|doi=10.1309/3G1XDX0DVHBNVKB4}}</ref>
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| *In September 2002, a consensus group at the Second International Workshop on WM in Athens, Greece, defined WM as a distinct clinicopathologic entity with characteristics of bone marrow infiltration associated with IgM monoclonal gammopathy by WM.<ref name="KonoplevMedeiros2005">{{cite journal|last1=Konoplev|first1=Sergej|last2=Medeiros|first2=L. Jeffrey|last3=Bueso-Ramos|first3=Carlos E.|last4=Jorgensen|first4=Jeffrey L.|last5=Lin|first5=Pei|title=Immunophenotypic Profile of Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia|journal=American Journal of Clinical Pathology|volume=124|issue=3|year=2005|pages=414–420|issn=0002-9173|doi=10.1309/3G1XDX0DVHBNVKB4}}</ref>
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| | ==[[Lymphoplasmacytic lymphoma classification|Classification]]== |
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| | ==[[Lymphoplasmacytic lymphoma pathophysiology|Pathophysiology]]== |
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| | ==[[Lymphoplasmacytic lymphoma causes|Causes]]== |
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| The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
| | ==[[Lymphoplasmacytic lymphoma differential diagnosis|Differentiating Lymphoplasmacytic lymphoma from other Diseases]]== |
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| In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
| | ==[[Lymphoplasmacytic lymphoma epidemiology and demographics|Epidemiology and Demographics]]== |
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| In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].
| | ==[[Lymphoplasmacytic lymphoma risk factors|Risk Factors]]== |
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| There have been several outbreaks of [disease name], including -----.
| | ==[[Lymphoplasmacytic lymphoma screening|Screening]]== |
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| In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].
| | ==[[Lymphoplasmacytic lymphoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Classification==
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| *There is no established system of classification for lymphoplasmacytic lymphoma (LPL).
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| [Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
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| [Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3].
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| [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
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| Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
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| If the staging system involves specific and characteristic findings and features:
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| According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
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| The staging of [malignancy name] is based on the [staging system].
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| There is no established system for the staging of [malignancy name].
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| ==Pathophysiology==
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| ===Multiple factors associated with pathogenesis===
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| The exact oncogenesis of WM has not been defined yet. However, several factors that play a role in pathogenesis of WM are listed as follow:
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| *History of WM/LPL or other B-cell disorders in first-degree relatives of patient with WM.<ref name="pmid16357024">{{cite journal| author=Treon SP, Hunter ZR, Aggarwal A, Ewen EP, Masota S, Lee C et al.| title=Characterization of familial Waldenstrom's macroglobulinemia. | journal=Ann Oncol | year= 2006 | volume= 17 | issue= 3 | pages= 488-94 | pmid=16357024 | doi=10.1093/annonc/mdj111 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16357024 }} </ref><ref name="pmid17785558">{{cite journal| author=McMaster ML, Csako G, Giambarresi TR, Vasquez L, Berg M, Saddlemire S et al.| title=Long-term evaluation of three multiple-case Waldenstrom macroglobulinemia families. | journal=Clin Cancer Res | year= 2007 | volume= 13 | issue= 17 | pages= 5063-9 | pmid=17785558 | doi=10.1158/1078-0432.CCR-07-0299 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17785558 }} </ref><ref name="pmid18703425">{{cite journal| author=Kristinsson SY, Björkholm M, Goldin LR, McMaster ML, Turesson I, Landgren O| title=Risk of lymphoproliferative disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population-based study in Sweden. | journal=Blood | year= 2008 | volume= 112 | issue= 8 | pages= 3052-6 | pmid=18703425 | doi=10.1182/blood-2008-06-162768 | pmc=2569164 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18703425 }} </ref>
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| *History of precursor condition of IgM Monoclonal gammopathy of undetermined significance (MGUS) in patient of WM.<ref name="pmid12720118">{{cite journal| author=Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML et al.| title=Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. | journal=Semin Oncol | year= 2003 | volume= 30 | issue= 2 | pages= 110-5 | pmid=12720118 | doi=10.1053/sonc.2003.50082 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12720118 }} </ref><ref name="pmid12881316">{{cite journal| author=Kyle RA, Therneau TM, Rajkumar SV, Remstein ED, Offord JR, Larson DR et al.| title=Long-term follow-up of IgM monoclonal gammopathy of undetermined significance. | journal=Blood | year= 2003 | volume= 102 | issue= 10 | pages= 3759-64 | pmid=12881316 | doi=10.1182/blood-2003-03-0801 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881316 }} </ref><ref name="pmid12881316">{{cite journal| author=Kyle RA, Therneau TM, Rajkumar SV, Remstein ED, Offord JR, Larson DR et al.| title=Long-term follow-up of IgM monoclonal gammopathy of undetermined significance. | journal=Blood | year= 2003 | volume= 102 | issue= 10 | pages= 3759-64 | pmid=12881316 | doi=10.1182/blood-2003-03-0801 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881316 }} </ref><ref name="pmid16034042">{{cite journal| author=Baldini L, Goldaniga M, Guffanti A, Broglia C, Cortelazzo S, Rossi A et al.| title=Immunoglobulin M monoclonal gammopathies of undetermined significance and indolent Waldenstrom's macroglobulinemia recognize the same determinants of evolution into symptomatic lymphoid disorders: proposal for a common prognostic scoring system. | journal=J Clin Oncol | year= 2005 | volume= 23 | issue= 21 | pages= 4662-8 | pmid=16034042 | doi=10.1200/JCO.2005.06.147 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16034042 }} </ref><ref name="pmid12881316">{{cite journal| author=Kyle RA, Therneau TM, Rajkumar SV, Remstein ED, Offord JR, Larson DR et al.| title=Long-term follow-up of IgM monoclonal gammopathy of undetermined significance. | journal=Blood | year= 2003 | volume= 102 | issue= 10 | pages= 3759-64 | pmid=12881316 | doi=10.1182/blood-2003-03-0801 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881316 }} </ref><ref name="pmid19182202">{{cite journal| author=Landgren O, Kristinsson SY, Goldin LR, Caporaso NE, Blimark C, Mellqvist UH et al.| title=Risk of plasma cell and lymphoproliferative disorders among 14621 first-degree relatives of 4458 patients with monoclonal gammopathy of undetermined significance in Sweden. | journal=Blood | year= 2009 | volume= 114 | issue= 4 | pages= 791-5 | pmid=19182202 | doi=10.1182/blood-2008-12-191676 | pmc=2716021 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19182202 }} </ref>
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| *Age older than 60 years.<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref>
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| *Personal and family history of autoimmune diseases with autoantibodies leads to 2-3 fold higher risk of developing WM, especially elevated risks are associated with following:
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| **Hepatitis C virus infection (overall 20-30% increased risk for non-Hodgkin lymohoma and 3-fold increased risk for WM).<ref name="pmid18703425">{{cite journal| author=Kristinsson SY, Björkholm M, Goldin LR, McMaster ML, Turesson I, Landgren O| title=Risk of lymphoproliferative disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population-based study in Sweden. | journal=Blood | year= 2008 | volume= 112 | issue= 8 | pages= 3052-6 | pmid=18703425 | doi=10.1182/blood-2008-06-162768 | pmc=2569164 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18703425 }} </ref><ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref><ref name="pmid18387498">{{cite journal| author=de Sanjose S, Benavente Y, Vajdic CM, Engels EA, Morton LM, Bracci PM et al.| title=Hepatitis C and non-Hodgkin lymphoma among 4784 cases and 6269 controls from the International Lymphoma Epidemiology Consortium. | journal=Clin Gastroenterol Hepatol | year= 2008 | volume= 6 | issue= 4 | pages= 451-8 | pmid=18387498 | doi=10.1016/j.cgh.2008.02.011 | pmc=3962672 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18387498 }} </ref>
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| **HIV.<ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref>
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| **Rickettsiosis.<ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref>
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| **Sjogren syndrome.<ref name="pmid20181958">{{cite journal| author=Kristinsson SY, Koshiol J, Björkholm M, Goldin LR, McMaster ML, Turesson I et al.| title=Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. | journal=J Natl Cancer Inst | year= 2010 | volume= 102 | issue= 8 | pages= 557-67 | pmid=20181958 | doi=10.1093/jnci/djq043 | pmc=2857799 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20181958 }} </ref><ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref>
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| **Autoimmune hemolytic anemia.<ref name="pmid20181958">{{cite journal| author=Kristinsson SY, Koshiol J, Björkholm M, Goldin LR, McMaster ML, Turesson I et al.| title=Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. | journal=J Natl Cancer Inst | year= 2010 | volume= 102 | issue= 8 | pages= 557-67 | pmid=20181958 | doi=10.1093/jnci/djq043 | pmc=2857799 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20181958 }} </ref><ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref>
| |
| **Sarcoidosis.<ref name="pmid16985251">{{cite journal| author=Landgren O, Engels EA, Pfeiffer RM, Gridley G, Mellemkjaer L, Olsen JH et al.| title=Autoimmunity and susceptibility to Hodgkin lymphoma: a population-based case-control study in Scandinavia. | journal=J Natl Cancer Inst | year= 2006 | volume= 98 | issue= 18 | pages= 1321-30 | pmid=16985251 | doi=10.1093/jnci/djj361 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16985251 }} </ref>
| |
| **SLE.<ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref>
| |
| *Hay fever.
| |
| *Male gender.<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref><ref name="pmid17488966">{{cite journal| author=Giordano TP, Henderson L, Landgren O, Chiao EY, Kramer JR, El-Serag H et al.| title=Risk of non-Hodgkin lymphoma and lymphoproliferative precursor diseases in US veterans with hepatitis C virus. | journal=JAMA | year= 2007 | volume= 297 | issue= 18 | pages= 2010-7 | pmid=17488966 | doi=10.1001/jama.297.18.2010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17488966 }} </ref>
| |
| *White race/ethnicity.<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref><ref name="pmid16150940">{{cite journal| author=Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS| title=Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001. | journal=Blood | year= 2006 | volume= 107 | issue= 1 | pages= 265-76 | pmid=16150940 | doi=10.1182/blood-2005-06-2508 | pmc=1895348 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16150940 }} </ref>
| |
| ===Environmental Factors===
| |
| According to some recent studies, exposure to following environmental factors seems to have association with the development of WM:<ref name="pmid20308603">{{cite journal| author=Royer RH, Koshiol J, Giambarresi TR, Vasquez LG, Pfeiffer RM, McMaster ML| title=Differential characteristics of Waldenström macroglobulinemia according to patterns of familial aggregation. | journal=Blood | year= 2010 | volume= 115 | issue= 22 | pages= 4464-71 | pmid=20308603 | doi=10.1182/blood-2009-10-247973 | pmc=2881498 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20308603 }} </ref><ref name="pmid25174029">{{cite journal| author=Vajdic CM, Landgren O, McMaster ML, Slager SL, Brooks-Wilson A, Smith A et al.| title=Medical history, lifestyle, family history, and occupational risk factors for lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia: the InterLymph Non-Hodgkin Lymphoma Subtypes Project. | journal=J Natl Cancer Inst Monogr | year= 2014 | volume= 2014 | issue= 48 | pages= 87-97 | pmid=25174029 | doi=10.1093/jncimonographs/lgu002 | pmc=4155457 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25174029 }} </ref>
| |
| *Occupational (Farming).
| |
| *Pesticides.
| |
| *Wood dust.
| |
| *Organic solvents.
| |
| ===Genetics===
| |
| There are many cytogenetic abnormalities & mutations associated with WM:
| |
| *MYD88 L265P somatic mutation (90%).<ref name="TreonXu2012">{{cite journal|last1=Treon|first1=Steven P.|last2=Xu|first2=Lian|last3=Yang|first3=Guang|last4=Zhou|first4=Yangsheng|last5=Liu|first5=Xia|last6=Cao|first6=Yang|last7=Sheehy|first7=Patricia|last8=Manning|first8=Robert J.|last9=Patterson|first9=Christopher J.|last10=Tripsas|first10=Christina|last11=Arcaini|first11=Luca|last12=Pinkus|first12=Geraldine S.|last13=Rodig|first13=Scott J.|last14=Sohani|first14=Aliyah R.|last15=Harris|first15=Nancy Lee|last16=Laramie|first16=Jason M.|last17=Skifter|first17=Donald A.|last18=Lincoln|first18=Stephen E.|last19=Hunter|first19=Zachary R.|title=MYD88 L265P Somatic Mutation in Waldenström's Macroglobulinemia|journal=New England Journal of Medicine|volume=367|issue=9|year=2012|pages=826–833|issn=0028-4793|doi=10.1056/NEJMoa1200710}}</ref><ref name="pmid23355535">{{cite journal| author=Varettoni M, Arcaini L, Zibellini S, Boveri E, Rattotti S, Riboni R et al.| title=Prevalence and clinical significance of the MYD88 (L265P) somatic mutation in Waldenstrom's macroglobulinemia and related lymphoid neoplasms. | journal=Blood | year= 2013 | volume= 121 | issue= 13 | pages= 2522-8 | pmid=23355535 | doi=10.1182/blood-2012-09-457101 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23355535 }} </ref><ref name="pmid26231802">{{cite journal| author=Shi M, Spurgeon S, Press R, Olson S, Fan G| title=MYD88 mutation analysis of a rare composite chronic lymphocyte leukemia and lymphoplasmacytic lymphoma by flow cytometry cell sorting. | journal=Ann Hematol | year= 2015 | volume= 94 | issue= 11 | pages= 1941-4 | pmid=26231802 | doi=10.1007/s00277-015-2460-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26231802 }} </ref><ref name="pmid23836557">{{cite journal| author=Yang G, Zhou Y, Liu X, Xu L, Cao Y, Manning RJ et al.| title=A mutation in MYD88 (L265P) supports the survival of lymphoplasmacytic cells by activation of Bruton tyrosine kinase in Waldenström macroglobulinemia. | journal=Blood | year= 2013 | volume= 122 | issue= 7 | pages= 1222-32 | pmid=23836557 | doi=10.1182/blood-2012-12-475111 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23836557 }} </ref><ref name="pmid21179087">{{cite journal| author=Ngo VN, Young RM, Schmitz R, Jhavar S, Xiao W, Lim KH et al.| title=Oncogenically active MYD88 mutations in human lymphoma. | journal=Nature | year= 2011 | volume= 470 | issue= 7332 | pages= 115-9 | pmid=21179087 | doi=10.1038/nature09671 | pmc=5024568 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21179087 }} </ref><ref name="pmid24224040">{{cite journal| author=Mori N, Ohwashi M, Yoshinaga K, Mitsuhashi K, Tanaka N, Teramura M et al.| title=L265P mutation of the MYD88 gene is frequent in Waldenström's macroglobulinemia and its absence in myeloma. | journal=PLoS One | year= 2013 | volume= 8 | issue= 11 | pages= e80088 | pmid=24224040 | doi=10.1371/journal.pone.0080088 | pmc=3818242 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24224040 }} </ref><ref name="pmid29080258">{{cite journal| author=Abeykoon JP, Paludo J, King RL, Ansell SM, Gertz MA, LaPlant BR et al.| title=MYD88 mutation status does not impact overall survival in Waldenström macroglobulinemia. | journal=Am J Hematol | year= 2018 | volume= 93 | issue= 2 | pages= 187-194 | pmid=29080258 | doi=10.1002/ajh.24955 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29080258 }} </ref>
| |
| *Deletions in 6q21-22.1 gene (50%).<ref name="TreonHunter2006">{{cite journal|last1=Treon|first1=S. P.|last2=Hunter|first2=Z. R.|last3=Aggarwal|first3=A.|last4=Ewen|first4=E. P.|last5=Masota|first5=S.|last6=Lee|first6=C.|last7=Santos|first7=D. Ditzel|last8=Hatjiharissi|first8=E.|last9=Xu|first9=L.|last10=Leleu|first10=X.|last11=Tournilhac|first11=O.|last12=Patterson|first12=C. J.|last13=Manning|first13=R.|last14=Branagan|first14=A. R.|last15=Morton|first15=C. C.|title=Characterization of familial Waldenström's macroglobulinemia|journal=Annals of Oncology|volume=17|issue=3|year=2006|pages=488–494|issn=1569-8041|doi=10.1093/annonc/mdj111}}</ref>
| |
| *Somatic hypermutation in IGHV.<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref>
| |
| *t(9;14)(p13;q32) (50%).<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref>
| |
| *C-X-C chemokine receptor type 4 (CXCR4) 5338X mutations (30-35%).<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref>
| |
| *Trisomy 4 (20%).<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref>
| |
| ===Histopathology===
| |
| WM/LPL is a form of an indolent (slowly growing) non-hodgkin lymphoma. LPL is called so because the lymphoma cells have the characteristics of both lymphocytes and plasma cells. After a detailed clinicopathological assessment and review of the published literature, the following diagnostic criteria was proposed for WM:<ref name="pmid12720135">{{cite journal| author=Owen RG| title=Developing diagnostic criteria in Waldenstrom's macroglobulinemia. | journal=Semin Oncol | year= 2003 | volume= 30 | issue= 2 | pages= 196-200 | pmid=12720135 | doi=10.1053/sonc.2003.50069 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12720135 }} </ref>
| |
| *IgM monoclonal gammopathy of any concentration.
| |
| *Bone marrow infiltration by small lymphocytes, plasmacytoid cells and plasma cells in following patterns:<ref name="pmid11554171">{{cite journal| author=Owen RG, Barrans SL, Richards SJ, O'Connor SJ, Child JA, Parapia LA et al.| title=Waldenström macroglobulinemia. Development of diagnostic criteria and identification of prognostic factors. | journal=Am J Clin Pathol | year= 2001 | volume= 116 | issue= 3 | pages= 420-8 | pmid=11554171 | doi=10.1309/4LCN-JMPG-5U71-UWQB | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11554171 }} </ref>
| |
| **Diffuse.
| |
| **Interstitial.
| |
| **Nodular.
| |
| **Paratrabecular.
| |
| *A surface Ig(+)CD19(+)CD20(+)CD5(-)CD10(-)CD23(-) immunophenotype.
| |
| *Following lymphoid organs are involved in WM:
| |
| **Bone marrow.
| |
| **Lymph nodes.
| |
| **Spleen.
| |
| *The bone marrow contains variable numbers of pleomorphic lymphoid cells.
| |
| *Dutcher bodies may be seen as intracytoplasmic inclusions positive for periodic acid Schiff.
| |
| *Mast cell hyperplasia is common and may stimulate tumor cell proliferation and monoclonal IgM secretion.
| |
| *Gene expression profiling has indicated that lymphoid cells of WM more closely resemble those of chronic lymphocytic leukemia than those of myeloma.<ref name="pmid16804116">{{cite journal| author=Chng WJ, Schop RF, Price-Troska T, Ghobrial I, Kay N, Jelinek DF et al.| title=Gene-expression profiling of Waldenstrom macroglobulinemia reveals a phenotype more similar to chronic lymphocytic leukemia than multiple myeloma. | journal=Blood | year= 2006 | volume= 108 | issue= 8 | pages= 2755-63 | pmid=16804116 | doi=10.1182/blood-2006-02-005488 | pmc=1895596 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16804116 }} </ref>
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|
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| OR
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| It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
| |
| | |
| OR
| |
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| [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
| |
| | |
| OR
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| Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
| |
| | |
| OR
| |
| | |
| | |
| [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
| |
| | |
| OR
| |
| | |
| The progression to [disease name] usually involves the [molecular pathway].
| |
| | |
| OR
| |
| | |
| The pathophysiology of [disease/malignancy] depends on the histological subtype.
| |
| | |
| ==Causes==
| |
| Disease name] may be caused by [cause1], [cause2], or [cause3].
| |
| | |
| OR
| |
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| Common causes of [disease] include [cause1], [cause2], and [cause3].
| |
| | |
| OR
| |
| | |
| The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
| |
| | |
| OR
| |
| | |
| The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click [[Pericarditis causes#Overview|here]].
| |
| | |
| ==Differentiating ((Page name)) from Other Diseases==
| |
| [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
| |
| | |
| OR
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| [Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
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| | |
| ==Epidemiology and Demographics==
| |
| *Non-Hodgkinís lymphomas (NHLs) are a very heterogeneous group of cancers that develop in the lymph nodes present throughout the body.
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| *There are over 25 subtypes of NHL, although most are diagnosed infrequently.
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| *Lymphoplasmacytic lymphoma is one of those rare subtypes, accounting for only 1 to 2% of all NHLs.
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| *This lymphoma arises from plasma cells, which are mature B-lymphocytes.
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| *It is most often associated with an increased IgM protein produced by the lymphoma cells and is commonly referred to as Waldenstrom’s macroglobulinemia (WM), after the Swedish physician who first described the syndrome in 1944.
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| *WM is considered an indolent lymphoma; the disease progresses very slowly, and patients tend to live many years after diagnosis.
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| *However, most patients are diagnosed with advanced disease, and although WM responds well to a variety of chemotherapy drugs, it is generally considered to be incurable.
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| *WM is somewhat more common in men compared to women, and it is much more common in white patients than in African-Americans.
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| *The average age at diagnosis is between 60 and 65 years.
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| The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
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| | |
| OR
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| In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
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| | |
| OR
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| In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.
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| Patients of all age groups may develop [disease name].
| |
| | |
| OR
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| The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
| |
| | |
| OR
| |
| | |
| [Disease name] commonly affects individuals younger than/older than [number of years] years of age.
| |
| | |
| OR
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| [Chronic disease name] is usually first diagnosed among [age group].
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| | |
| OR
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| [Acute disease name] commonly affects [age group].
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| There is no racial predilection to [disease name].
| |
| | |
| OR
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| [Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
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| | |
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| [Disease name] affects men and women equally.
| |
| | |
| OR
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| [Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
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| | |
| | |
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| The majority of [disease name] cases are reported in [geographical region].
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| | |
| OR
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| [Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].
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| | |
| ==Risk Factors==
| |
| *History of WM/LPL or other B-cell disorders in first-degree relatives of patient with WM.<ref name="pmid16357024">{{cite journal| author=Treon SP, Hunter ZR, Aggarwal A, Ewen EP, Masota S, Lee C et al.| title=Characterization of familial Waldenstrom's macroglobulinemia. | journal=Ann Oncol | year= 2006 | volume= 17 | issue= 3 | pages= 488-94 | pmid=16357024 | doi=10.1093/annonc/mdj111 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16357024 }} </ref><ref name="pmid17785558">{{cite journal| author=McMaster ML, Csako G, Giambarresi TR, Vasquez L, Berg M, Saddlemire S et al.| title=Long-term evaluation of three multiple-case Waldenstrom macroglobulinemia families. | journal=Clin Cancer Res | year= 2007 | volume= 13 | issue= 17 | pages= 5063-9 | pmid=17785558 | doi=10.1158/1078-0432.CCR-07-0299 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17785558 }} </ref><ref name="pmid18703425">{{cite journal| author=Kristinsson SY, Björkholm M, Goldin LR, McMaster ML, Turesson I, Landgren O| title=Risk of lymphoproliferative disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population-based study in Sweden. | journal=Blood | year= 2008 | volume= 112 | issue= 8 | pages= 3052-6 | pmid=18703425 | doi=10.1182/blood-2008-06-162768 | pmc=2569164 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18703425 }} </ref>
| |
| *History of precursor condition of IgM Monoclonal gammopathy of undetermined significance (MGUS) in patient of WM.<ref name="pmid12720118">{{cite journal| author=Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML et al.| title=Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. | journal=Semin Oncol | year= 2003 | volume= 30 | issue= 2 | pages= 110-5 | pmid=12720118 | doi=10.1053/sonc.2003.50082 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12720118 }} </ref><ref name="pmid12881316">{{cite journal| author=Kyle RA, Therneau TM, Rajkumar SV, Remstein ED, Offord JR, Larson DR et al.| title=Long-term follow-up of IgM monoclonal gammopathy of undetermined significance. | journal=Blood | year= 2003 | volume= 102 | issue= 10 | pages= 3759-64 | pmid=12881316 | doi=10.1182/blood-2003-03-0801 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881316 }} </ref><ref name="pmid12881316">{{cite journal| author=Kyle RA, Therneau TM, Rajkumar SV, Remstein ED, Offord JR, Larson DR et al.| title=Long-term follow-up of IgM monoclonal gammopathy of undetermined significance. | journal=Blood | year= 2003 | volume= 102 | issue= 10 | pages= 3759-64 | pmid=12881316 | doi=10.1182/blood-2003-03-0801 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881316 }} </ref><ref name="pmid16034042">{{cite journal| author=Baldini L, Goldaniga M, Guffanti A, Broglia C, Cortelazzo S, Rossi A et al.| title=Immunoglobulin M monoclonal gammopathies of undetermined significance and indolent Waldenstrom's macroglobulinemia recognize the same determinants of evolution into symptomatic lymphoid disorders: proposal for a common prognostic scoring system. | journal=J Clin Oncol | year= 2005 | volume= 23 | issue= 21 | pages= 4662-8 | pmid=16034042 | doi=10.1200/JCO.2005.06.147 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16034042 }} </ref><ref name="pmid12881316">{{cite journal| author=Kyle RA, Therneau TM, Rajkumar SV, Remstein ED, Offord JR, Larson DR et al.| title=Long-term follow-up of IgM monoclonal gammopathy of undetermined significance. | journal=Blood | year= 2003 | volume= 102 | issue= 10 | pages= 3759-64 | pmid=12881316 | doi=10.1182/blood-2003-03-0801 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881316 }} </ref><ref name="pmid19182202">{{cite journal| author=Landgren O, Kristinsson SY, Goldin LR, Caporaso NE, Blimark C, Mellqvist UH et al.| title=Risk of plasma cell and lymphoproliferative disorders among 14621 first-degree relatives of 4458 patients with monoclonal gammopathy of undetermined significance in Sweden. | journal=Blood | year= 2009 | volume= 114 | issue= 4 | pages= 791-5 | pmid=19182202 | doi=10.1182/blood-2008-12-191676 | pmc=2716021 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19182202 }} </ref>
| |
| *Age older than 60 years.<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref>
| |
| *Personal and family history of autoimmune diseases with autoantibodies leads to 2-3 fold higher risk of developing WM, especially elevated risks are associated with following:
| |
| **Hepatitis C virus infection (overall 20-30% increased risk for non-Hodgkin lymohoma and 3-fold increased risk for WM).<ref name="pmid18703425">{{cite journal| author=Kristinsson SY, Björkholm M, Goldin LR, McMaster ML, Turesson I, Landgren O| title=Risk of lymphoproliferative disorders among first-degree relatives of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia patients: a population-based study in Sweden. | journal=Blood | year= 2008 | volume= 112 | issue= 8 | pages= 3052-6 | pmid=18703425 | doi=10.1182/blood-2008-06-162768 | pmc=2569164 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18703425 }} </ref><ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref><ref name="pmid18387498">{{cite journal| author=de Sanjose S, Benavente Y, Vajdic CM, Engels EA, Morton LM, Bracci PM et al.| title=Hepatitis C and non-Hodgkin lymphoma among 4784 cases and 6269 controls from the International Lymphoma Epidemiology Consortium. | journal=Clin Gastroenterol Hepatol | year= 2008 | volume= 6 | issue= 4 | pages= 451-8 | pmid=18387498 | doi=10.1016/j.cgh.2008.02.011 | pmc=3962672 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18387498 }} </ref>
| |
| **HIV.<ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref>
| |
| **Rickettsiosis.<ref name="pmid18809818">{{cite journal| author=Koshiol J, Gridley G, Engels EA, McMaster ML, Landgren O| title=Chronic immune stimulation and subsequent Waldenström macroglobulinemia. | journal=Arch Intern Med | year= 2008 | volume= 168 | issue= 17 | pages= 1903-9 | pmid=18809818 | doi=10.1001/archinternmed.2008.4 | pmc=2670401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18809818 }} </ref>
| |
| **Sjogren syndrome.<ref name="pmid20181958">{{cite journal| author=Kristinsson SY, Koshiol J, Björkholm M, Goldin LR, McMaster ML, Turesson I et al.| title=Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. | journal=J Natl Cancer Inst | year= 2010 | volume= 102 | issue= 8 | pages= 557-67 | pmid=20181958 | doi=10.1093/jnci/djq043 | pmc=2857799 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20181958 }} </ref><ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref>
| |
| **Autoimmune hemolytic anemia.<ref name="pmid20181958">{{cite journal| author=Kristinsson SY, Koshiol J, Björkholm M, Goldin LR, McMaster ML, Turesson I et al.| title=Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. | journal=J Natl Cancer Inst | year= 2010 | volume= 102 | issue= 8 | pages= 557-67 | pmid=20181958 | doi=10.1093/jnci/djq043 | pmc=2857799 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20181958 }} </ref><ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref>
| |
| **Sarcoidosis.<ref name="pmid16985251">{{cite journal| author=Landgren O, Engels EA, Pfeiffer RM, Gridley G, Mellemkjaer L, Olsen JH et al.| title=Autoimmunity and susceptibility to Hodgkin lymphoma: a population-based case-control study in Scandinavia. | journal=J Natl Cancer Inst | year= 2006 | volume= 98 | issue= 18 | pages= 1321-30 | pmid=16985251 | doi=10.1093/jnci/djj361 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16985251 }} </ref>
| |
| **SLE.<ref name="pmid18263783">{{cite journal| author=Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J et al.| title=Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. | journal=Blood | year= 2008 | volume= 111 | issue= 8 | pages= 4029-38 | pmid=18263783 | doi=10.1182/blood-2007-10-119974 | pmc=2288717 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18263783 }} </ref>
| |
| *Hay fever.
| |
| *Male gender.<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref><ref name="pmid17488966">{{cite journal| author=Giordano TP, Henderson L, Landgren O, Chiao EY, Kramer JR, El-Serag H et al.| title=Risk of non-Hodgkin lymphoma and lymphoproliferative precursor diseases in US veterans with hepatitis C virus. | journal=JAMA | year= 2007 | volume= 297 | issue= 18 | pages= 2010-7 | pmid=17488966 | doi=10.1001/jama.297.18.2010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17488966 }} </ref>
| |
| *White race/ethnicity.<ref name="pmid28366781">{{cite journal| author=Yun S, Johnson AC, Okolo ON, Arnold SJ, McBride A, Zhang L et al.| title=Waldenström Macroglobulinemia: Review of Pathogenesis and Management. | journal=Clin Lymphoma Myeloma Leuk | year= 2017 | volume= 17 | issue= 5 | pages= 252-262 | pmid=28366781 | doi=10.1016/j.clml.2017.02.028 | pmc=5413391 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28366781 }} </ref><ref name="pmid16150940">{{cite journal| author=Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS| title=Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001. | journal=Blood | year= 2006 | volume= 107 | issue= 1 | pages= 265-76 | pmid=16150940 | doi=10.1182/blood-2005-06-2508 | pmc=1895348 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16150940 }} </ref>
| |
| ===Environmental Factors===
| |
| According to some recent studies, exposure to following environmental factors seems to have association with the development of WM:<ref name="pmid20308603">{{cite journal| author=Royer RH, Koshiol J, Giambarresi TR, Vasquez LG, Pfeiffer RM, McMaster ML| title=Differential characteristics of Waldenström macroglobulinemia according to patterns of familial aggregation. | journal=Blood | year= 2010 | volume= 115 | issue= 22 | pages= 4464-71 | pmid=20308603 | doi=10.1182/blood-2009-10-247973 | pmc=2881498 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20308603 }} </ref><ref name="pmid25174029">{{cite journal| author=Vajdic CM, Landgren O, McMaster ML, Slager SL, Brooks-Wilson A, Smith A et al.| title=Medical history, lifestyle, family history, and occupational risk factors for lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia: the InterLymph Non-Hodgkin Lymphoma Subtypes Project. | journal=J Natl Cancer Inst Monogr | year= 2014 | volume= 2014 | issue= 48 | pages= 87-97 | pmid=25174029 | doi=10.1093/jncimonographs/lgu002 | pmc=4155457 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25174029 }} </ref>
| |
| *Occupational (Farming).
| |
| *Pesticides.
| |
| *Wood dust.
| |
| *Organic solvents.
| |
| | |
| There are no established risk factors for [disease name].
| |
| | |
| OR
| |
| | |
| The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
| |
| | |
| OR
| |
| | |
| Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
| |
| | |
| OR
| |
| | |
| Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.
| |
| | |
| ==Screening==
| |
| There is insufficient evidence to recommend routine screening for [disease/malignancy].
| |
| | |
| OR
| |
| | |
| According to the [guideline name], screening for [disease name] is not recommended.
| |
| | |
| OR
| |
| | |
| According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].
| |
| | |
| ==Natural History, Complications, and Prognosis==
| |
| If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
| |
| | |
| OR
| |
| | |
| Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
| |
| | |
| OR
| |
| | |
| Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
| |
|
| |
|
| ==Diagnosis== | | ==Diagnosis== |
| ===Diagnostic Study of Choice===
| |
| The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
| |
|
| |
|
| OR
| | [[Lymphoplasmacytic lymphoma diagnostic study of choice|Diagnostic study of choice]] | [[Lymphoplasmacytic lymphoma history and symptoms|History and Symptoms]] | [[Lymphoplasmacytic lymphoma physical examination|Physical Examination]] | [[Lymphoplasmacytic lymphoma laboratory findings|Laboratory Findings]] | [[Lymphoplasmacytic lymphoma electrocardiogram|Electrocardiogram]] | [[Lymphoplasmacytic lymphoma x ray|X-Ray Findings]] | [[Lymphoplasmacytic lymphoma echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Lymphoplasmacytic lymphoma CT scan|CT-Scan Findings]] | [[Lymphoplasmacytic lymphoma MRI|MRI Findings]] | [[Lymphoplasmacytic lymphoma other imaging findings|Other Imaging Findings]] | [[Lymphoplasmacytic lymphoma other diagnostic studies|Other Diagnostic Studies]] |
| | |
| The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
| |
| | |
| OR
| |
| | |
| The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
| |
| | |
| OR
| |
| | |
| There are no established criteria for the diagnosis of [disease name].
| |
| | |
| ===History and Symptoms===
| |
| *Mucous membrane bleeding (oronasal).
| |
| *Fatigue
| |
| *Weakness.
| |
| *The lymphoma cells make varying amounts of a protein called immunoglobulin M (IgM, or macroglobulin). Higher amounts of this protein than normal in blood tends to make it thick leading to hyperviscosity syndrome (when blood becomes thick, it is harder for blood to flow through small blood vessels), and when this occurs, the condition is termed as Waldenstrom macroglobulinemia. This excess amount of IgM antibodies can be ultimately associated with circulatory problems leading to less blood flow to the brain, the eyes or other organs, which may cause any of the following:
| |
| **Dizziness.
| |
| **Headache.
| |
| **Blurred vision or loss of vision.
| |
| **Shortness of breath.
| |
| **Numbness and tingling of the fingers or toes (called peripheral neuropathy).
| |
| **Muscle weakness.
| |
| **Confusion.
| |
| **Anemia (IgM binds to RBCs).
| |
| **Kidney disease (IgM deposits in kidney tissues).
| |
| **Cryoglobulinemia and vasculitis (inflammation of small blood vessels occurs when IgM antibody forms immune complexes with cold exposure).
| |
| **Congestive heart failure.
| |
| *Most people with lymphoplasmacytic lymphoma do not have B symptoms (unexplained fever, drenching night sweats and unexplained weight loss).
| |
| | |
| | |
| | |
| Read more: http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/more-types-of-nhl/lymphoplasmacytic-lymphoma/?region=on#ixzz5eavoTzUH
| |
| The majority of patients with [disease name] are asymptomatic.
| |
| | |
| OR
| |
| | |
| The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
| |
| | |
| ===Physical Examination===
| |
| Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
| |
| | |
| OR
| |
| | |
| Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
| |
| | |
| OR
| |
| | |
| The presence of [finding(s)] on physical examination is diagnostic of [disease name].
| |
| | |
| OR
| |
| | |
| The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
| |
| | |
| ===Laboratory Findings===
| |
| An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
| |
| | |
| OR
| |
| | |
| Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
| |
| | |
| OR
| |
| | |
| [Test] is usually normal among patients with [disease name].
| |
| | |
| OR
| |
| | |
| Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
| |
| | |
| OR
| |
| | |
| There are no diagnostic laboratory findings associated with [disease name].
| |
| | |
| ===Electrocardiogram===
| |
| There are no ECG findings associated with [disease name].
| |
| | |
| OR
| |
| | |
| An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
| |
| | |
| ===X-ray===
| |
| There are no x-ray findings associated with [disease name].
| |
| | |
| OR
| |
| | |
| An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
| |
| | |
| OR
| |
| | |
| There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
| |
| | |
| ===Echocardiography or Ultrasound===
| |
| There are no echocardiography/ultrasound findings associated with [disease name].
| |
| | |
| OR
| |
| | |
| Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3]. | |
| | |
| OR
| |
| | |
| There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
| |
| | |
| ===CT scan===
| |
| There are no CT scan findings associated with [disease name].
| |
| | |
| OR
| |
| | |
| [Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
| |
| | |
| OR
| |
| | |
| There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
| |
| | |
| ===MRI===
| |
| There are no MRI findings associated with [disease name].
| |
| | |
| OR
| |
| | |
| [Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
| |
| | |
| OR
| |
| | |
| There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
| |
| | |
| ===Other Imaging Findings===
| |
| There are no other imaging findings associated with [disease name].
| |
| | |
| OR
| |
| | |
| [Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
| |
| | |
| ===Other Diagnostic Studies===
| |
| There are no other diagnostic studies associated with [disease name].
| |
| | |
| OR
| |
| | |
| [Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
| |
| | |
| OR
| |
| | |
| Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].
| |
|
| |
|
| ==Treatment== | | ==Treatment== |
| One or more of the following treatments can be given for lymphoplasmacytic lymphoma.
| | [[Lymphoplasmacytic lymphoma medical therapy|Medical Therapy]] | [[Lymphoplasmacytic lymphoma surgery|Surgery]] | [[Lymphoplasmacytic lymphoma primary prevention|Primary Prevention]] | [[Lymphoplasmacytic lymphoma secondary prevention|Secondary Prevention]] | [[Lymphoplasmacytic lymphoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Lymphoplasmacytic lymphoma future or investigational therapies|Future or Investigational Therapies]] |
| ===Watchful waiting===
| |
| Watchful waiting (also called active surveillance) may be offered for lymphoplasmacytic lymphoma because it develops slowly and may not need to be treated right away. The healthcare team will carefully monitor the person with lymphoplasmacytic lymphoma and start treatment when symptoms appear, such as hyperviscosity syndrome, or there are signs that the disease is progressing more quickly.
| |
| ===Chemotherapy===
| |
| *People with lymphoplasmacytic lymphoma who have symptoms or hyperviscosity syndrome are usually given chemotherapy. Chemotherapy drugs that may be used with or without prednisone include:
| |
| **Chlorambucil (Leukeran)
| |
| **Fludarabine (Fludara)
| |
| **Bendamustine (Treanda)
| |
| **Cyclophosphamide (Cytoxan, Procytox)
| |
| | |
| *Combinations of chemotherapy drugs that may be used include:
| |
| **DRC – dexamethasone (Decadron, Dexasone), rituximab (Rituxan) and cyclophosphamide
| |
| **BRD – bortezomib (Velcade) and rituximab, with or without dexamethasone
| |
| **CVP – cyclophosphamide, vincristine (Oncovin) and prednisone
| |
| **R-CVP – CVP with rituximab
| |
| **Thalidomide (Thalomid) and rituximab
| |
| | |
| ===Targeted therapy===
| |
| *Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.
| |
| *Targeted therapy drugs used alone or in combination to treat lymphoplasmacytic lymphoma include rituximab, bortezomib and ibrutinib (Imbruvica).
| |
| ===Immunotherapy===
| |
| *Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunomodulatory drugs are a type of immunotherapy that interferes with the growth and division of cancer cells.
| |
| *Thalidomide is a type of immunomodulatory drug that may be used to treat lymphoplasmacytic lymphoma.
| |
| ===Radiation therapy===
| |
| External beam radiation therapy may be used to treat lymphoplasmacytic lymphoma that develops outside of the lymphatic system (called extralymphatic disease), but this is rare.
| |
| ===Stem cell transplant===
| |
| *Some people with lymphoplasmacytic lymphoma may be offered a stem cell transplant.
| |
| *It may be used if the lymphoma comes back (recurs) after treatment or doesn’t respond to other treatments (called refractory disease).
| |
| *Many people with lymphoplasmacytic lymphoma are older or may not be in good health, so a stem cell transplant may not be a good treatment option for them.
| |
| | |
| | |
| Read more: http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/more-types-of-nhl/lymphoplasmacytic-lymphoma/?region=on#ixzz5eb6iT7G6
| |
| ===Medical Therapy===
| |
| There is no treatment for [disease name]; the mainstay of therapy is supportive care.
| |
| | |
| OR
| |
| | |
| Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
| |
| | |
| OR
| |
| | |
| The majority of cases of [disease name] are self-limited and require only supportive care.
| |
| | |
| OR
| |
| | |
| [Disease name] is a medical emergency and requires prompt treatment.
| |
| | |
| OR
| |
| | |
| The mainstay of treatment for [disease name] is [therapy].
| |
| | |
| OR
| |
|
| |
| The optimal therapy for [malignancy name] depends on the stage at diagnosis.
| |
| | |
| OR
| |
| | |
| [Therapy] is recommended among all patients who develop [disease name].
| |
| | |
| OR
| |
| | |
| Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
| |
| | |
| OR
| |
| | |
| Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
| |
| | |
| OR
| |
| | |
| Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
| |
| | |
| OR
| |
| | |
| Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
| |
| | |
| ===Surgery===
| |
| Surgical intervention is not recommended for the management of [disease name].
| |
| | |
| OR
| |
| | |
| Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]
| |
| | |
| OR
| |
| | |
| The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
| |
| | |
| OR
| |
| | |
| The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
| |
| | |
| OR
| |
| | |
| Surgery is the mainstay of treatment for [disease or malignancy].
| |
| | |
| ===Primary Prevention===
| |
| There are no established measures for the primary prevention of [disease name].
| |
| | |
| OR
| |
| | |
| There are no available vaccines against [disease name].
| |
| | |
| OR
| |
| | |
| Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
| |
| | |
| OR
| |
| | |
| [Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].
| |
| | |
| ===Secondary Prevention===
| |
| There are no established measures for the secondary prevention of [disease name].
| |
| | |
| OR
| |
|
| |
|
| Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].
| | ==Case Studies== |
| | [[Lymphoplasmacytic lymphoma case study one|Case #1]] |
|
| |
|
| ==References==
| | [[Category: (name of the system)]] |
| {{reflist|2}}
| |