Lymphoplasmacytic lymphoma natural history, complications and prognosis: Difference between revisions

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==Overview==
==Overview==
If left untreated, patients with [[asymptomatic]] disease may progress to develop [[fatigue]], [[weight loss]], [[peripheral neuropathy]] and other [[symptoms]] of the disease. Common [[complications]] of [[lymphoplasmacytic lymphoma]] include: [[hyperviscosity syndrome]], [[Cold agglutinin disease|cold haemagglutinin disease]], [[cryoglobulinemia]], [[peripheral neuropathy]], [[primary amyloidosis]], [[Renal insufficiency|renal insufficiency,]] malabsorptive [[Diarrhea|diarrhea]], and [[Blurred vision|visual abnormalities]]. [[Prognosis]] varies depending on the various factors involved. Five year [[survival rate]] is 87% for low-risk [[disease]] and 36% for high-risk [[disease]].
If left untreated, [[patients]] with [[asymptomatic]] [[disease]] may progress to [[Development|develop]] [[fatigue]], [[weight loss]], [[peripheral neuropathy]], [[shortness of breath]], [[purpura]], [[raynaud's phenomenon]], and [[vision problems]]. Common [[complications]] of [[lymphoplasmacytic lymphoma]] include: [[hyperviscosity syndrome]], [[Cold agglutinin disease|cold haemagglutinin disease]], [[cryoglobulinemia]], [[peripheral neuropathy]], [[primary amyloidosis]], [[Renal insufficiency|renal insufficiency,]] [[Malabsorption|malabsorptive]] [[Diarrhea|diarrhea]], [[Blurred vision|visual abnormalities]], [[congestive heart failure]], and [[schnitzler syndrome]]. Late and [[rare]] severe [[complications]] include [[Richter's transformation|richter syndrome]], and [[bing-Neel syndrome]]. [[Prognosis]] varies [[Dependent variable|depending]] on the various factors involved. [[Five year survival rate]] is 87% for low-[[RiskMetrics|risk]] [[disease]] and 36% for high-[[RiskMetrics|risk]] [[disease]]. A [[Standardized moment|standardized]] [[Scoring rule|scoring]] [[system]] known as the International [[Prognostic]] [[Staging (pathology)|Staging]] [[System]] for [[Waldenström's macroglobulinemia|Waldenström's Macroglobulinemia]] (IPSSWM) [[Risk stratification tools|risk stratifies]] the [[patients]] with [[Waldenström's macroglobulinemia|Waldenstrom's macroglobulinemia]].  


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
=== Natural History: ===
=== Natural History ===
====Initial symptoms====
====Initial symptoms====
*The symptoms of [[lymphoplasmacytic lymphoma]] usually develop in the seventh and eighth decade of life.
*[[Symptoms]] of [[lymphoplasmacytic lymphoma]] usually [[Development|develop]] in the seventh and eighth decade of [[life]]
*It will typically start with [[symptoms]] such as:
*It will typically start with [[symptoms]] such as:
**[[Fatigue]]
**[[Fatigue]]
**Unexplained weight loss
**Unexplained [[weight loss]]
**[[Numbness]] and [[tingling]] associated with [[peripheral neuropathy]]
**[[Numbness]] and [[tingling]] [[Association (statistics)|associated]] with [[peripheral neuropathy]]
**[[Dyspnea|Shortness of breath]]
**[[Dyspnea|Shortness of breath]]
**[[Purpura]]
**[[Purpura]]
**[[Raynaud's phenomenon]]
**[[Raynaud's phenomenon]]
**[[Blurred vision|Vision problems]] such as [[blurred vision]], [[vision loss]] or blind spots<ref name="pmid22139816">{{cite journal| author=Wang H, Chen Y, Li F, Delasalle K, Wang J, Alexanian R et al.| title=Temporal and geographic variations of Waldenstrom macroglobulinemia incidence: a large population-based study. | journal=Cancer | year= 2012 | volume= 118 | issue= 15 | pages= 3793-800 | pmid=22139816 | doi=10.1002/cncr.26627 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22139816  }} </ref>
**[[Blurred vision|Vision problems]] such as [[blurred vision]], [[vision loss]] or [[Blind spot (vision)|blind spots]]<ref name="pmid22139816">{{cite journal| author=Wang H, Chen Y, Li F, Delasalle K, Wang J, Alexanian R et al.| title=Temporal and geographic variations of Waldenstrom macroglobulinemia incidence: a large population-based study. | journal=Cancer | year= 2012 | volume= 118 | issue= 15 | pages= 3793-800 | pmid=22139816 | doi=10.1002/cncr.26627 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22139816  }} </ref>


==== Complications ====
==== Complications ====
* As the [[disease]] progresses, the following [[complications]] can commonly occur in [[patients]] with [[lymphoplasmacytic lymphoma]]:
* As the [[disease]] progresses, the following [[complications]] can commonly occur in [[patients]] with [[lymphoplasmacytic lymphoma]]:
** [[Hyperviscosity syndrome|Hyperviscosity syndrome]]<ref name="pmid11736938">{{cite journal| author=García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A et al.| title=Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases. | journal=Br J Haematol | year= 2001 | volume= 115 | issue= 3 | pages= 575-82 | pmid=11736938 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11736938  }} </ref>
**[[Hyperviscosity syndrome|Hyperviscosity syndrome]]<ref name="pmid11736938">{{cite journal| author=García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A et al.| title=Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases. | journal=Br J Haematol | year= 2001 | volume= 115 | issue= 3 | pages= 575-82 | pmid=11736938 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11736938  }} </ref>
** [[Cryoglobulinemia|Cryoglobulinemia]]<ref name="pmid14871241">{{cite journal| author=Michael AB, Lawes M, Kamalarajan M, Huissoon A, Pratt G| title=Cryoglobulinaemia as an acute presentation of Waldenstrom's macroglobulinaemia. | journal=Br J Haematol | year= 2004 | volume= 124 | issue= 5 | pages= 565 | pmid=14871241 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14871241  }} </ref>
** [[Cryoglobulinemia|Cryoglobulinemia]]<ref name="pmid14871241">{{cite journal| author=Michael AB, Lawes M, Kamalarajan M, Huissoon A, Pratt G| title=Cryoglobulinaemia as an acute presentation of Waldenstrom's macroglobulinaemia. | journal=Br J Haematol | year= 2004 | volume= 124 | issue= 5 | pages= 565 | pmid=14871241 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14871241  }} </ref>
** [[Peripheral neuropathy]](15%)<ref name="pmid16421127">{{cite journal| author=Levine T, Pestronk A, Florence J, Al-Lozi MT, Lopate G, Miller T et al.| title=Peripheral neuropathies in Waldenström's macroglobulinaemia. | journal=J Neurol Neurosurg Psychiatry | year= 2006 | volume= 77 | issue= 2 | pages= 224-8 | pmid=16421127 | doi=10.1136/jnnp.2005.071175 | pmc=2077569 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16421127  }} </ref>
** [[Peripheral neuropathy]](15%)<ref name="pmid16421127">{{cite journal| author=Levine T, Pestronk A, Florence J, Al-Lozi MT, Lopate G, Miller T et al.| title=Peripheral neuropathies in Waldenström's macroglobulinaemia. | journal=J Neurol Neurosurg Psychiatry | year= 2006 | volume= 77 | issue= 2 | pages= 224-8 | pmid=16421127 | doi=10.1136/jnnp.2005.071175 | pmc=2077569 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16421127  }} </ref>
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** [[Fundoscopy|Fundoscopic]] [[abnormalities]]<ref name="pmid11736938" />
** [[Fundoscopy|Fundoscopic]] [[abnormalities]]<ref name="pmid11736938" />
**[[Congestive heart failure]]
**[[Congestive heart failure]]
**[[Schnitzler syndrome]] which is an [[autoimmune]] [[Complication (medicine)|complication]] associated with elevated [[IgM]] levels, that leads to [[fever]], [[itchy skin]] [[lesions]], and [[joint aches]].
**[[Schnitzler syndrome]] which is an [[autoimmune]] [[Complication (medicine)|complication]] [[Association (statistics)|associated]] with elevated [[IgM]] levels, that [[Lead|leads]] to [[fever]], [[itchy skin]] [[lesions]], and [[joint aches]]


==== Late and rare severe complications ====
==== Late and rare severe complications ====
*Large cell [[Transformation (genetics)|transformation]] (Richter [[syndrome]]):
*[[Large cell]] [[Transformation (genetics)|transformation]] ([[Richter's transformation|Richter syndrome]]):
**Dr. Richter of the University of Minnesota first recognized the blockage in maturity of the [[lymphoma]] cells at a point when they can't mature beyond the large cell stage thus, leading to large cell [[Transformation (genetics)|transformation]].
**[[Doctor of Medicine|Dr]]. [[Richter's transformation|Richter]] of the [[University of Minnesota Medical Center|University of Minnesota]] first [[Recognition sequence|recognized]] the blockage in [[Maturity (psychological)|maturity]] of the [[lymphoma]] [[Cells (biology)|cells]] at a point when they can't [[Maturity (psychological)|mature]] beyond the [[large cell]] [[Stages of human development|stage]] thus, [[Lead|leading]] to [[large cell]] [[Transformation (genetics)|transformation]]
*[[Central nervous system]] [[Lymphoma]] ([[Bing-Neel syndrome]]):<ref name="pmid9686227">{{cite journal| author=Civit T, Coulbois S, Baylac F, Taillandier L, Auque J| title=[Waldenström's macroglobulinemia and cerebral lymphoplasmocytic proliferation: Bing and Neel syndrome. Apropos of a new case]. | journal=Neurochirurgie | year= 1997 | volume= 43 | issue= 4 | pages= 245-9 | pmid=9686227 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9686227  }} </ref>
*[[Central nervous system]] [[Lymphoma]] ([[Bing-Neel syndrome]]):<ref name="pmid9686227">{{cite journal| author=Civit T, Coulbois S, Baylac F, Taillandier L, Auque J| title=[Waldenström's macroglobulinemia and cerebral lymphoplasmocytic proliferation: Bing and Neel syndrome. Apropos of a new case]. | journal=Neurochirurgie | year= 1997 | volume= 43 | issue= 4 | pages= 245-9 | pmid=9686227 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9686227  }} </ref>
**The development of [[Waldenström macroglobulinemia]] cells in the [[central nervous system]] was first described by Drs. Bing and Neel and carries their names as the [[Bing-Neel syndrome]]. WM involves [[CNS]] in following two forms:
**The [[Development (biology)|development]] of [[Waldenström macroglobulinemia]] [[Cells (biology)|cells]] in the [[central nervous system]] was first described by [[Doctor of Medicine|Drs]]. [[Bing–Neel syndrome|Bing]] and Neel and [[Carrying capacity|carries]] their names as the [[Bing-Neel syndrome]]. [[Waldenström's macroglobulinemia|WM]] involves [[CNS]] in following two forms:
***Actual [[tumor]] developing in the [[brain]] substance causing [[seizures]] and [[paralysis]].
***Actual [[tumor]] [[Development (biology)|developing]] in the [[brain]] [[substance]] [[Causes|causing]] [[seizures]] and [[paralysis]]
***[[Tumor]] cells invading [[meninges]] and [[cranial nerves]] without causing the actual [[tumors]] and with or without [[CSF]] [[cryoglobulinemia]],<ref name="pmid19362988">{{cite journal| author=Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH| title=Bing-Neel Syndrome revisited. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 1 | pages= 104-6 | pmid=19362988 | doi=10.3816/CLM.2009.n.028 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19362988  }} </ref> leading to following [[Symptom|symptoms]]:<ref name="pmid19951888">{{cite journal| author=Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG| title=Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 6 | pages= 462-6 | pmid=19951888 | doi=10.3816/CLM.2009.n.091 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19951888  }} </ref>
***[[Tumor cell|Tumor cells]] [[Invasive|invading]] [[meninges]] and [[cranial nerves]] without [[Causes|causing]] the actual [[tumors]] and with or without [[CSF]] [[cryoglobulinemia]],<ref name="pmid19362988">{{cite journal| author=Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH| title=Bing-Neel Syndrome revisited. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 1 | pages= 104-6 | pmid=19362988 | doi=10.3816/CLM.2009.n.028 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19362988  }} </ref> [[Lead|leading]] to the following [[Symptom|symptoms]]:<ref name="pmid19951888">{{cite journal| author=Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG| title=Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 6 | pages= 462-6 | pmid=19951888 | doi=10.3816/CLM.2009.n.091 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19951888  }} </ref>
****[[Headache]]  
****[[Headache]]
****[[Confusion]]
****[[Confusion]]
****[[Neck stiffness]]
****[[Neck stiffness]]
****Sporadic loss of [[Motor skill|motor]] [[Function (biology)|function]]  
****Sporadic loss of [[Motor skill|motor]] [[Function (biology)|function]]
****[[Facial paralysis]]
****[[Facial paralysis]]
****[[Drooping eyelid]]
****[[Drooping eyelid]]
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====Summary of natural history and complications of lymphoplasmacytic lymphoma====
====Summary of natural history and complications of lymphoplasmacytic lymphoma====
{{Family tree/start}}
{{Family tree/start}}
{{Family tree | | | | A01 | | | |A01= '''Initial symptoms''':
{{Family tree |boxstyle=width: 200px; text-align: left;| | A01 | |A01= '''Initial symptoms'''
*[[Fatigue]]
*[[Fatigue]]
*Unexplained [[weight loss]]
*Unexplained [[weight loss]]
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*[[Raynaud's phenomenon]]
*[[Raynaud's phenomenon]]
*[[Blurred vision|Vision problems]] such as [[blurred vision]], [[vision loss]] or blind spots}}
*[[Blurred vision|Vision problems]] such as [[blurred vision]], [[vision loss]] or blind spots}}
{{Family tree | | | | |!| | | | | }}
{{Family tree | | |!| | }}
{{Family tree | | | | B01 | | | |B01= '''Common [[complications]]''':
{{Family tree |boxstyle=text-align: left;| | B01 | |B01= '''Common complications'''
*[[Hyperviscosity syndrome|Hyperviscosity syndrome.]]
*[[Hyperviscosity syndrome|Hyperviscosity syndrome]]
*[[Cryoglobulinemia|Cryoglobulinemia.]]
*[[Cryoglobulinemia|Cryoglobulinemia]]
*[[Peripheral neuropathy]](15%)
*[[Peripheral neuropathy]] (15%)
*[[Amyloidosis]] of the [[heart]], [[kidney]], [[liver]], [[lungs]], and [[Joints|joints.]]
*[[Amyloidosis]] of the [[heart]], [[kidney]], [[liver]], [[lungs]], and [[Joints|joints]]
*[[Cold agglutinin disease|Cold haemagglutinin disease]]/[[Autoimmune hemolytic anemia]](<10%)
*[[Cold agglutinin disease|Cold haemagglutinin disease]]/[[Autoimmune hemolytic anemia]] (<10%)
*[[Malabsorption|Gastrointestinal malabsorption.]]
*[[Malabsorption|Gastrointestinal malabsorption]]
*[[Renal insufficiency|Renal insufficiency.]]
*[[Renal insufficiency|Renal insufficiency]]
* [[Fundoscopy|Fundoscopic]] [[abnormalities]]
*[[Fundoscopy|Fundoscopic]] [[abnormalities]]
*[[Congestive heart failure]]
*[[Congestive heart failure]]
*[[Schnitzler syndrome]] which is an [[autoimmune]] [[Complication (medicine)|complication]] associated with elevated [[IgM]] levels, that leads to [[fever]], itchy [[skin]] lesions, and [[joint aches]]}}
*[[Schnitzler syndrome]] which is associated with:
{{Family tree | | | | |!| | | | | }}
:*Elevated [[IgM]] levels
{{Family tree | | | | C01 | | | |C01= '''Late and rare severe [[complications]]''':
:*[[Fever]]
:*Itchy [[skin]] lesions
:*[[Joint aches]]}}
{{Family tree | | |!| |}}
{{Family tree |boxstyle=text-align: left;| | C01 | |C01= '''Late and rare severe complications'''
*Large cell [[Transformation (genetics)|transformation]] (Richter [[syndrome]])
*Large cell [[Transformation (genetics)|transformation]] (Richter [[syndrome]])
*[[Central nervous system]] [[Lymphoma]] ([[Bing-Neel syndrome]])}}
*[[Central nervous system]] [[Lymphoma]] ([[Bing-Neel syndrome]])}}
{{Family tree/end}}
{{Family tree/end}}


=== Prognosis: ===
=== Prognosis ===
*[[Prognosis]] is generally poor.
*[[Prognosis]] is [[Generality|generally]] poor
*The median survival from the time of [[diagnosis]] is 6.4 years.<ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*The [[median]] [[Survival analysis|survival]] from the [[Time series|time]] of [[diagnosis]] is 6.4 [[Year|years]]<ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*The median [[disease]]-specific survival is 11.2 years.<ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*The [[median]] [[disease]]-[[Specific activity|specific]] [[Survival analysis|survival]] is 11.2 [[Year|years]]<ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*Approximately 10% [[patients]] still live at 15 years.<ref name="MorelDuhamel2009">{{cite journal|last1=Morel|first1=P.|last2=Duhamel|first2=A.|last3=Gobbi|first3=P.|last4=Dimopoulos|first4=M. A.|last5=Dhodapkar|first5=M. V.|last6=McCoy|first6=J.|last7=Crowley|first7=J.|last8=Ocio|first8=E. M.|last9=Garcia-Sanz|first9=R.|last10=Treon|first10=S. P.|last11=Leblond|first11=V.|last12=Kyle|first12=R. A.|last13=Barlogie|first13=B.|last14=Merlini|first14=G.|title=International prognostic scoring system for Waldenstrom macroglobulinemia|journal=Blood|volume=113|issue=18|year=2009|pages=4163–4170|issn=0006-4971|doi=10.1182/blood-2008-08-174961}}</ref><ref name="pmid10792277">{{cite journal| author=Kyle RA, Greipp PR, Gertz MA, Witzig TE, Lust JA, Lacy MQ et al.| title=Waldenström's macroglobulinaemia: a prospective study comparing daily with intermittent oral chlorambucil. | journal=Br J Haematol | year= 2000 | volume= 108 | issue= 4 | pages= 737-42 | pmid=10792277 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10792277  }} </ref>
*Approximately 10% [[patients]] still [[Life|live]] uptil 15 [[Year|years]]<ref name="MorelDuhamel2009">{{cite journal|last1=Morel|first1=P.|last2=Duhamel|first2=A.|last3=Gobbi|first3=P.|last4=Dimopoulos|first4=M. A.|last5=Dhodapkar|first5=M. V.|last6=McCoy|first6=J.|last7=Crowley|first7=J.|last8=Ocio|first8=E. M.|last9=Garcia-Sanz|first9=R.|last10=Treon|first10=S. P.|last11=Leblond|first11=V.|last12=Kyle|first12=R. A.|last13=Barlogie|first13=B.|last14=Merlini|first14=G.|title=International prognostic scoring system for Waldenstrom macroglobulinemia|journal=Blood|volume=113|issue=18|year=2009|pages=4163–4170|issn=0006-4971|doi=10.1182/blood-2008-08-174961}}</ref><ref name="pmid10792277">{{cite journal| author=Kyle RA, Greipp PR, Gertz MA, Witzig TE, Lust JA, Lacy MQ et al.| title=Waldenström's macroglobulinaemia: a prospective study comparing daily with intermittent oral chlorambucil. | journal=Br J Haematol | year= 2000 | volume= 108 | issue= 4 | pages= 737-42 | pmid=10792277 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10792277  }} </ref>
*5-year [[survival rate]] is 78%.
*5-[[year]] [[survival rate]] is 78%
*In the last decade (2001-2010), the median overall survival for all LPL groups has improved to just over 8 years compared to 6 years in the previous decade (1991-2000).
*In the last decade (2001-2010), the [[median]] overall [[Survival analysis|survival]] for all LPL [[Group (sociology)|groups]] has [[Improving agent|improved]] to just over 8 [[Year|years]] [[Comparability|compared]] to 6 [[Year|years]] in the previous decade (1991-2000)
*Cases without [[MYD88]] [[mutations]] are associated with [[adenopathy]] and a worse outcome.<ref name="pmid24553177">{{cite journal| author=Treon SP, Cao Y, Xu L, Yang G, Liu X, Hunter ZR| title=Somatic mutations in MYD88 and CXCR4 are determinants of clinical presentation and overall survival in Waldenstrom macroglobulinemia. | journal=Blood | year= 2014 | volume= 123 | issue= 18 | pages= 2791-6 | pmid=24553177 | doi=10.1182/blood-2014-01-550905 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24553177  }} </ref>  
*[[Case-based reasoning|Cases]] without [[MYD88]] [[mutations]] are [[Association (statistics)|associated]] with [[adenopathy]] and a worse [[outcome]]<ref name="pmid24553177">{{cite journal| author=Treon SP, Cao Y, Xu L, Yang G, Liu X, Hunter ZR| title=Somatic mutations in MYD88 and CXCR4 are determinants of clinical presentation and overall survival in Waldenstrom macroglobulinemia. | journal=Blood | year= 2014 | volume= 123 | issue= 18 | pages= 2791-6 | pmid=24553177 | doi=10.1182/blood-2014-01-550905 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24553177  }} </ref>
*After 2000, a 2-fold increased [[mortality]] is reported in [[patients]] diagnosed with LPL when compared with expected [[population]] [[mortality]].<ref name="KyleLarson2018">{{cite journal|last1=Kyle|first1=Robert A.|last2=Larson|first2=Dirk R.|last3=McPhail|first3=Ellen D.|last4=Therneau|first4=Terry M.|last5=Dispenzieri|first5=Angela|last6=Kumar|first6=Shaji|last7=Kapoor|first7=Prashant|last8=Cerhan|first8=James R.|last9=Rajkumar|first9=S. Vincent|title=Fifty-Year Incidence of Waldenström Macroglobulinemia in Olmsted County, Minnesota, From 1961 Through 2010: A Population-Based Study With Complete Case Capture and Hematopathologic Review|journal=Mayo Clinic Proceedings|volume=93|issue=6|year=2018|pages=739–746|issn=00256196|doi=10.1016/j.mayocp.2018.02.011}}</ref>
*After 2000, a 2-fold increased [[mortality]] is [[Reporting disease cases|reported]] in [[patients]] [[Diagnosis|diagnosed]] with LPL when [[Comparability|compared]] with [[Expected value|expected]] [[population]] [[mortality]]<ref name="KyleLarson2018">{{cite journal|last1=Kyle|first1=Robert A.|last2=Larson|first2=Dirk R.|last3=McPhail|first3=Ellen D.|last4=Therneau|first4=Terry M.|last5=Dispenzieri|first5=Angela|last6=Kumar|first6=Shaji|last7=Kapoor|first7=Prashant|last8=Cerhan|first8=James R.|last9=Rajkumar|first9=S. Vincent|title=Fifty-Year Incidence of Waldenström Macroglobulinemia in Olmsted County, Minnesota, From 1961 Through 2010: A Population-Based Study With Complete Case Capture and Hematopathologic Review|journal=Mayo Clinic Proceedings|volume=93|issue=6|year=2018|pages=739–746|issn=00256196|doi=10.1016/j.mayocp.2018.02.011}}</ref>
*The presence of [[symptoms]] is associated with a particularly poor [[prognosis]] among patients with the disease.
*The [[Presenting symptom|presence]] of [[symptoms]] is [[Association (statistics)|associated]] with a particularly poor [[prognosis]] among [[patients]] with the [[disease]]
*[[Prognosis]] of [[asymptomatic]] [[patients]] is similar to that of the general [[population]] with a 10-year [[survival rate]] of 70-75%.<ref name="pmid20702770">{{cite journal| author=Ansell SM, Kyle RA, Reeder CB, Fonseca R, Mikhael JR, Morice WG et al.| title=Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines. | journal=Mayo Clin Proc | year= 2010 | volume= 85 | issue= 9 | pages= 824-33 | pmid=20702770 | doi=10.4065/mcp.2010.0304 | pmc=2931618 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20702770  }} </ref><ref name="pmid15756000">{{cite journal| author=Gobbi PG, Baldini L, Broglia C, Goldaniga M, Comelli M, Morel P et al.| title=Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance? | journal=Clin Cancer Res | year= 2005 | volume= 11 | issue= 5 | pages= 1786-90 | pmid=15756000 | doi=10.1158/1078-0432.CCR-04-1899 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15756000  }} </ref>
*[[Prognosis]] of [[asymptomatic]] [[patients]] is [[Similarity matrix|similar]] to that of the [[Generalization|general]] [[population]] with a 10-[[year]] [[survival rate]] of 70-75%<ref name="pmid20702770">{{cite journal| author=Ansell SM, Kyle RA, Reeder CB, Fonseca R, Mikhael JR, Morice WG et al.| title=Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines. | journal=Mayo Clin Proc | year= 2010 | volume= 85 | issue= 9 | pages= 824-33 | pmid=20702770 | doi=10.4065/mcp.2010.0304 | pmc=2931618 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20702770  }} </ref><ref name="pmid15756000">{{cite journal| author=Gobbi PG, Baldini L, Broglia C, Goldaniga M, Comelli M, Morel P et al.| title=Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance? | journal=Clin Cancer Res | year= 2005 | volume= 11 | issue= 5 | pages= 1786-90 | pmid=15756000 | doi=10.1158/1078-0432.CCR-04-1899 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15756000  }} </ref>


===Adverse prognostic factors:===
===Adverse prognostic factors===
*Some of the pretreatment factors associated with shorter survival in LPL [[patients]] are:<ref name="pmid12881396">{{cite journal| author=Dimopoulos MA, Hamilos G, Zervas K, Symeonidis A, Kouvatseas G, Roussou P et al.| title=Survival and prognostic factors after initiation of treatment in Waldenstrom's macroglobulinemia. | journal=Ann Oncol | year= 2003 | volume= 14 | issue= 8 | pages= 1299-305 | pmid=12881396 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881396  }} </ref><ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*Some of the pretreatment factors associated with shorter [[Survival analysis|survival]] in LPL [[patients]] are:<ref name="pmid12881396">{{cite journal| author=Dimopoulos MA, Hamilos G, Zervas K, Symeonidis A, Kouvatseas G, Roussou P et al.| title=Survival and prognostic factors after initiation of treatment in Waldenstrom's macroglobulinemia. | journal=Ann Oncol | year= 2003 | volume= 14 | issue= 8 | pages= 1299-305 | pmid=12881396 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881396  }} </ref><ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
**[[Age]] >/=65 years
**[[Clinical]] [[Parameter|parameters]]:
**[[Organomegaly]] ([[Hepatosplenomegaly]])
***[[Age]] >/=65 [[Year|years]]
**[[B symptoms]] ([[weight loss]], [[fever]] or [[night sweats]])
***[[Hyperviscosity]] [[symptoms]]
**[[Anemia]] (Hb < 10.0 g/dl)
***[[Organomegaly|Bulky organomegaly]] ([[Hepatosplenomegaly]])
**[[Platelets]] <100 x 10(6)/dl
***[[Bulking agents|Bulky]] [[lymphadenopathy]]
**[[Albumin]] <3.5 g/dl
***[[B symptoms]] ([[weight loss]], [[fever]] or [[night sweats]])
**[[Bone marrow]] lymphoplasmacytic infiltrate >/=50%
***[[Presenting symptom|Presence]] of [[symptomatic]] or unresponsive [[neuropathy]]
**Elevated beta2-microglobulin ( associated with 3-fold increase in death)
***[[Hemolytic anemia]]
**[[Leucopenia]] (<4.0 x 10(9)/l)
**[[Medical laboratory|Laboratory]] [[Parameter|parameters]]:<ref name="pmid25325033">{{cite journal| author=Yoo C, Yoon DH, Suh C| title=Serum beta-2 microglobulin in malignant lymphomas: an old but powerful prognostic factor. | journal=Blood Res | year= 2014 | volume= 49 | issue= 3 | pages= 148-53 | pmid=25325033 | doi=10.5045/br.2014.49.3.148 | pmc=4188779 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25325033  }}</ref><ref name="pmid4558655">{{cite journal| author=Peterson PA, Cunningham BA, Berggård I, Edelman GM| title=2 -Microglobulin--a free immunoglobulin domain. | journal=Proc Natl Acad Sci U S A | year= 1972 | volume= 69 | issue= 7 | pages= 1697-701 | pmid=4558655 | doi=10.1073/pnas.69.7.1697 | pmc=426781 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4558655  }}</ref>
**[[Thrombocytopenia]] (<150 x 10(9)/l)
***[[Hemoglobin]] < 10.0 g/dl)
**[[Quantitative]] [[IgM]] < 0.4 g/l
***[[Platelets]] <100 x 10(6)/dl
**[[Hyperviscosity]]
***[[Albumin]] <3.5 g/dl
*Other [[prognostic]] factors recently studied are:
***[[Bone marrow]] lymphoplasmacytic [[Infiltration (medical)|infiltrate]] >/=50%
**[[Serum]] free [[light chain]]<ref name="pmid18452095">{{cite journal| author=Leleu X, Moreau AS, Weller E, Roccaro AM, Coiteux V, Manning R et al.| title=Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia. | journal=Leuk Lymphoma | year= 2008 | volume= 49 | issue= 6 | pages= 1104-7 | pmid=18452095 | doi=10.1080/10428190802074619 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18452095  }} </ref>
***Elevated β2 microglobulin ([[Association (statistics)|associated]] with 3-fold increase in [[Death Cap|death]])
**[[Serum]] [[lactate dehydrogenase]]<ref name="pmid19362972">{{cite journal| author=Kastritis E, Zervas K, Repoussis P, Michali E, Katodrytou E, Zomas A et al.| title=Prognostication in young and old patients with Waldenström's macroglobulinemia: importance of the International Prognostic Scoring System and of serum lactate dehydrogenase. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 1 | pages= 50-2 | pmid=19362972 | doi=10.3816/CLM.2009.n.012 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19362972  }} </ref>
***[[Leucopenia]] (<4.0 x 10(9)/l)
**[[Serum]] [[soluble]] [[CD27]]<ref name="pmid18216294">{{cite journal| author=Ho AW, Hatjiharissi E, Ciccarelli BT, Branagan AR, Hunter ZR, Leleu X et al.| title=CD27-CD70 interactions in the pathogenesis of Waldenstrom macroglobulinemia. | journal=Blood | year= 2008 | volume= 112 | issue= 12 | pages= 4683-9 | pmid=18216294 | doi=10.1182/blood-2007-04-084525 | pmc=2597134 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18216294  }} </ref>
***[[Thrombocytopenia]] (<150 x 10(9)/l)
*Most of the [[prognostic]] factors have defined the [[outcome]] of [[lymphoplasmacytic lymphoma]] in [[patients]] requiring treatment. However, very few studies have evaluated the [[prognostic]] factors in [[patients]] who don't initially need the treatment.
***[[Quantitative]] [[IgM]] < 0.4 g/l
***[[Serum]] free [[light chain]]<ref name="pmid18452095">{{cite journal| author=Leleu X, Moreau AS, Weller E, Roccaro AM, Coiteux V, Manning R et al.| title=Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia. | journal=Leuk Lymphoma | year= 2008 | volume= 49 | issue= 6 | pages= 1104-7 | pmid=18452095 | doi=10.1080/10428190802074619 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18452095  }} </ref>
***[[Serum]] [[lactate dehydrogenase]]<ref name="pmid19362972">{{cite journal| author=Kastritis E, Zervas K, Repoussis P, Michali E, Katodrytou E, Zomas A et al.| title=Prognostication in young and old patients with Waldenström's macroglobulinemia: importance of the International Prognostic Scoring System and of serum lactate dehydrogenase. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 1 | pages= 50-2 | pmid=19362972 | doi=10.3816/CLM.2009.n.012 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19362972  }} </ref>
***[[Serum]] [[soluble]] [[CD27]]<ref name="pmid18216294">{{cite journal| author=Ho AW, Hatjiharissi E, Ciccarelli BT, Branagan AR, Hunter ZR, Leleu X et al.| title=CD27-CD70 interactions in the pathogenesis of Waldenstrom macroglobulinemia. | journal=Blood | year= 2008 | volume= 112 | issue= 12 | pages= 4683-9 | pmid=18216294 | doi=10.1182/blood-2007-04-084525 | pmc=2597134 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18216294  }} </ref>
*Most of the [[prognostic]] factors have defined the [[outcome]] of [[lymphoplasmacytic lymphoma]] in [[patients]] requiring [[Treatments|treatment]], however, very few [[Study design|studies]] have evaluated the [[prognostic]] factors in [[patients]] who don't initially need the [[Treatments|treatment]]


===Risk Stratification Criteria===
===Risk Stratification Criteria===
All the above [[prognostic]] data has been combined to risk stratify the WM patients and to formulate a standardized scoring system known as ''the International Prognostic Staging System for Waldenström's Macroglobulinemia'' (IPSSWM):<ref name="pmid19196866">{{cite journal| author=Morel P, Duhamel A, Gobbi P, Dimopoulos MA, Dhodapkar MV, McCoy J et al.| title=International prognostic scoring system for Waldenstrom macroglobulinemia. | journal=Blood | year= 2009 | volume= 113 | issue= 18 | pages= 4163-70 | pmid=19196866 | doi=10.1182/blood-2008-08-174961 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19196866  }} </ref>
All the above [[prognostic]] [[data]] has been [[Combinational logic|combined]] to [[Risk stratification tools|risk stratify]] the [[Waldenström's macroglobulinemia|WM]] [[patients]] and to [[Formulation|formulate]] a [[Standardized moment|standardized]] [[Scoring rule|scoring]] [[system]] known as '''the International [[Prognostic]] [[Staging (pathology)|Staging]] [[System]] for [[Waldenström's macroglobulinemia|Waldenström's Macroglobulinemia]] (IPSSWM)''':<ref name="pmid19196866">{{cite journal| author=Morel P, Duhamel A, Gobbi P, Dimopoulos MA, Dhodapkar MV, McCoy J et al.| title=International prognostic scoring system for Waldenstrom macroglobulinemia. | journal=Blood | year= 2009 | volume= 113 | issue= 18 | pages= 4163-70 | pmid=19196866 | doi=10.1182/blood-2008-08-174961 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19196866  }} </ref>


{| class="wikitable"
{| class="wikitable"
Line 119: Line 126:
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Score
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Score
|-
|-
| style="background:#DCDCDC;" align="center" + |Age > 65
! style="background:#DCDCDC;" align="center" + |[[Age]] > 65
| style="background:#F5F5F5;" align="center" + |1
| style="background:#F5F5F5;" align="center" + |1
|-
|-
| style="background:#DCDCDC;" align="center" + |Hemoglobin ≤ 11.5g/dl
! style="background:#DCDCDC;" align="center" + |[[Hemoglobin]] ≤ 11.5g/dl
| style="background:#F5F5F5;" align="center" + |1
| style="background:#F5F5F5;" align="center" + |1
|-
|-
| style="background:#DCDCDC;" align="center" + |Platelet ≤ 100,000μl
! style="background:#DCDCDC;" align="center" + |[[Platelet]] ≤ 100,000μl
| style="background:#F5F5F5;" align="center" + |1
| style="background:#F5F5F5;" align="center" + |1
|-
|-
| style="background:#DCDCDC;" align="center" + |β-microglobulin > 3mg/l
! style="background:#DCDCDC;" align="center" + |β-microglobulin > 3mg/l
| style="background:#F5F5F5;" align="center" + |1
| style="background:#F5F5F5;" align="center" + |1
|-
|-
| style="background:#DCDCDC;" align="center" + |IgM > 70g/l
! style="background:#DCDCDC;" align="center" + |[[Immunoglobulin M|IgM]] > 70g/l
| style="background:#F5F5F5;" align="center" + |1
| style="background:#F5F5F5;" align="center" + |1
|}
|}
Line 141: Line 148:
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Median survival
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Median survival
|-
|-
| style="background:#DCDCDC;" align="center" + |Low
! style="background:#DCDCDC;" align="center" + |Low
| style="background:#F5F5F5;" align="center" + |0-1 (except age)
| style="background:#F5F5F5;" align="center" + |0-1 (except age)
| style="background:#F5F5F5;" align="center" + |87%
| style="background:#F5F5F5;" align="center" + |87%
| style="background:#F5F5F5;" align="center" + |12 years
| style="background:#F5F5F5;" align="center" + |12 years
|-
|-
| style="background:#DCDCDC;" align="center" + |Intermediate
! style="background:#DCDCDC;" align="center" + |Intermediate
| style="background:#F5F5F5;" align="center" + |2 or age>65
| style="background:#F5F5F5;" align="center" + |2 or age>65
| style="background:#F5F5F5;" align="center" + |68%
| style="background:#F5F5F5;" align="center" + |68%
| style="background:#F5F5F5;" align="center" + |8 years
| style="background:#F5F5F5;" align="center" + |8 years
|-
|-
| style="background:#DCDCDC;" align="center" + |High
! style="background:#DCDCDC;" align="center" + |High
| style="background:#F5F5F5;" align="center" + |≥3
| style="background:#F5F5F5;" align="center" + |≥3
| style="background:#F5F5F5;" align="center" + |36%
| style="background:#F5F5F5;" align="center" + |36%

Latest revision as of 16:50, 29 October 2019

Lymphoplasmacytic lymphoma Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Overview

If left untreated, patients with asymptomatic disease may progress to develop fatigue, weight loss, peripheral neuropathy, shortness of breath, purpura, raynaud's phenomenon, and vision problems. Common complications of lymphoplasmacytic lymphoma include: hyperviscosity syndrome, cold haemagglutinin disease, cryoglobulinemia, peripheral neuropathy, primary amyloidosis, renal insufficiency, malabsorptive diarrhea, visual abnormalities, congestive heart failure, and schnitzler syndrome. Late and rare severe complications include richter syndrome, and bing-Neel syndrome. Prognosis varies depending on the various factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease. A standardized scoring system known as the International Prognostic Staging System for Waldenström's Macroglobulinemia (IPSSWM) risk stratifies the patients with Waldenstrom's macroglobulinemia.

Natural History, Complications, and Prognosis

Natural History

Initial symptoms

Complications

Late and rare severe complications

Summary of natural history and complications of lymphoplasmacytic lymphoma

 
Initial symptoms
 
 
 
 
 
 
 
 
Common complications
 
 
 
 
 
 
 
Late and rare severe complications
 

Prognosis

Adverse prognostic factors

Risk Stratification Criteria

All the above prognostic data has been combined to risk stratify the WM patients and to formulate a standardized scoring system known as the International Prognostic Staging System for Waldenström's Macroglobulinemia (IPSSWM):[25]

Risk factors Score
Age > 65 1
Hemoglobin ≤ 11.5g/dl 1
Platelet ≤ 100,000μl 1
β-microglobulin > 3mg/l 1
IgM > 70g/l 1
International prognostic scoring system for Waldenström macroglobulinemia
Risk group Score 5-year survival Median survival
Low 0-1 (except age) 87% 12 years
Intermediate 2 or age>65 68% 8 years
High ≥3 36% 3.5 years

References

  1. ↑ Wang H, Chen Y, Li F, Delasalle K, Wang J, Alexanian R; et al. (2012). "Temporal and geographic variations of Waldenstrom macroglobulinemia incidence: a large population-based study". Cancer. 118 (15): 3793–800. doi:10.1002/cncr.26627. PMID 22139816.
  2. ↑ 2.0 2.1 García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A; et al. (2001). "Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases". Br J Haematol. 115 (3): 575–82. PMID 11736938.
  3. ↑ Michael AB, Lawes M, Kamalarajan M, Huissoon A, Pratt G (2004). "Cryoglobulinaemia as an acute presentation of Waldenstrom's macroglobulinaemia". Br J Haematol. 124 (5): 565. PMID 14871241.
  4. ↑ Levine T, Pestronk A, Florence J, Al-Lozi MT, Lopate G, Miller T; et al. (2006). "Peripheral neuropathies in Waldenström's macroglobulinaemia". J Neurol Neurosurg Psychiatry. 77 (2): 224–8. doi:10.1136/jnnp.2005.071175. PMC 2077569. PMID 16421127.
  5. ↑ Zimmermann I, Gloor HJ, Rüttimann S (2001). "[General AL-amyloidosis: a rare complication in Waldenstrom macroglobulinemia]". Praxis (Bern 1994) (in German). 90 (47): 2050–5. PMID 11763619.
  6. ↑ Owen RG, Pratt G, Auer RL, Flatley R, Kyriakou C, Lunn MP; et al. (2014). "Guidelines on the diagnosis and management of Waldenström macroglobulinaemia". Br J Haematol. 165 (3): 316–33. doi:10.1111/bjh.12760. PMID 24528152.
  7. ↑ Veloso FT, Fraga J, Saleiro JV (1988). "Macroglobulinemia and small intestinal disease. A case report with review of the literature". J Clin Gastroenterol. 10 (5): 546–50. PMID 3141496.
  8. ↑ Vos JM, Gustine J, Rennke HG, Hunter Z, Manning RJ, Dubeau TE; et al. (2016). "Renal disease related to Waldenström macroglobulinaemia: incidence, pathology and clinical outcomes". Br J Haematol. 175 (4): 623–630. doi:10.1111/bjh.14279. PMID 27468978.
  9. ↑ Civit T, Coulbois S, Baylac F, Taillandier L, Auque J (1997). "[Waldenström's macroglobulinemia and cerebral lymphoplasmocytic proliferation: Bing and Neel syndrome. Apropos of a new case]". Neurochirurgie. 43 (4): 245–9. PMID 9686227.
  10. ↑ Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH (2009). "Bing-Neel Syndrome revisited". Clin Lymphoma Myeloma. 9 (1): 104–6. doi:10.3816/CLM.2009.n.028. PMID 19362988.
  11. ↑ Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG (2009). "Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options". Clin Lymphoma Myeloma. 9 (6): 462–6. doi:10.3816/CLM.2009.n.091. PMID 19951888.
  12. ↑ 12.0 12.1 12.2 Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM; et al. (2006). "Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia". Br J Haematol. 133 (2): 158–64. doi:10.1111/j.1365-2141.2006.06003.x. PMID 16611306.
  13. ↑ Morel, P.; Duhamel, A.; Gobbi, P.; Dimopoulos, M. A.; Dhodapkar, M. V.; McCoy, J.; Crowley, J.; Ocio, E. M.; Garcia-Sanz, R.; Treon, S. P.; Leblond, V.; Kyle, R. A.; Barlogie, B.; Merlini, G. (2009). "International prognostic scoring system for Waldenstrom macroglobulinemia". Blood. 113 (18): 4163–4170. doi:10.1182/blood-2008-08-174961. ISSN 0006-4971.
  14. ↑ Kyle RA, Greipp PR, Gertz MA, Witzig TE, Lust JA, Lacy MQ; et al. (2000). "Waldenström's macroglobulinaemia: a prospective study comparing daily with intermittent oral chlorambucil". Br J Haematol. 108 (4): 737–42. PMID 10792277.
  15. ↑ Treon SP, Cao Y, Xu L, Yang G, Liu X, Hunter ZR (2014). "Somatic mutations in MYD88 and CXCR4 are determinants of clinical presentation and overall survival in Waldenstrom macroglobulinemia". Blood. 123 (18): 2791–6. doi:10.1182/blood-2014-01-550905. PMID 24553177.
  16. ↑ Kyle, Robert A.; Larson, Dirk R.; McPhail, Ellen D.; Therneau, Terry M.; Dispenzieri, Angela; Kumar, Shaji; Kapoor, Prashant; Cerhan, James R.; Rajkumar, S. Vincent (2018). "Fifty-Year Incidence of Waldenström Macroglobulinemia in Olmsted County, Minnesota, From 1961 Through 2010: A Population-Based Study With Complete Case Capture and Hematopathologic Review". Mayo Clinic Proceedings. 93 (6): 739–746. doi:10.1016/j.mayocp.2018.02.011. ISSN 0025-6196.
  17. ↑ Ansell SM, Kyle RA, Reeder CB, Fonseca R, Mikhael JR, Morice WG; et al. (2010). "Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines". Mayo Clin Proc. 85 (9): 824–33. doi:10.4065/mcp.2010.0304. PMC 2931618. PMID 20702770.
  18. ↑ Gobbi PG, Baldini L, Broglia C, Goldaniga M, Comelli M, Morel P; et al. (2005). "Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance?". Clin Cancer Res. 11 (5): 1786–90. doi:10.1158/1078-0432.CCR-04-1899. PMID 15756000.
  19. ↑ Dimopoulos MA, Hamilos G, Zervas K, Symeonidis A, Kouvatseas G, Roussou P; et al. (2003). "Survival and prognostic factors after initiation of treatment in Waldenstrom's macroglobulinemia". Ann Oncol. 14 (8): 1299–305. PMID 12881396.
  20. ↑ Yoo C, Yoon DH, Suh C (2014). "Serum beta-2 microglobulin in malignant lymphomas: an old but powerful prognostic factor". Blood Res. 49 (3): 148–53. doi:10.5045/br.2014.49.3.148. PMC 4188779. PMID 25325033.
  21. ↑ Peterson PA, Cunningham BA, Berggård I, Edelman GM (1972). "2 -Microglobulin--a free immunoglobulin domain". Proc Natl Acad Sci U S A. 69 (7): 1697–701. doi:10.1073/pnas.69.7.1697. PMC 426781. PMID 4558655.
  22. ↑ Leleu X, Moreau AS, Weller E, Roccaro AM, Coiteux V, Manning R; et al. (2008). "Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia". Leuk Lymphoma. 49 (6): 1104–7. doi:10.1080/10428190802074619. PMID 18452095.
  23. ↑ Kastritis E, Zervas K, Repoussis P, Michali E, Katodrytou E, Zomas A; et al. (2009). "Prognostication in young and old patients with Waldenström's macroglobulinemia: importance of the International Prognostic Scoring System and of serum lactate dehydrogenase". Clin Lymphoma Myeloma. 9 (1): 50–2. doi:10.3816/CLM.2009.n.012. PMID 19362972.
  24. ↑ Ho AW, Hatjiharissi E, Ciccarelli BT, Branagan AR, Hunter ZR, Leleu X; et al. (2008). "CD27-CD70 interactions in the pathogenesis of Waldenstrom macroglobulinemia". Blood. 112 (12): 4683–9. doi:10.1182/blood-2007-04-084525. PMC 2597134. PMID 18216294.
  25. ↑ Morel P, Duhamel A, Gobbi P, Dimopoulos MA, Dhodapkar MV, McCoy J; et al. (2009). "International prognostic scoring system for Waldenstrom macroglobulinemia". Blood. 113 (18): 4163–70. doi:10.1182/blood-2008-08-174961. PMID 19196866.

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