Waldenström's macroglobulinemia
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| Waldenström macroglobulinemia Classification and external resources | |
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| Waldenström's macroglobulinemia. Bilateral involvement of orbit and eyelid. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology | |
| ICD-10 | C88.0 |
| ICD-9 | 273.3 |
| ICD-O: | M9761/3 |
| OMIM | 153600 |
| DiseasesDB | 14030 |
| MedlinePlus | 000588 |
| eMedicine | med/2395 |
| MeSH | D008258 |
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Overview
Waldenström macroglobulinemia (WM) is cancer involving a subtype of white blood cells called lymphocytes. The main attributing antibody is IgM. It is a type of lymphoproliferative disease, and shares clinical characteristics with the indolent non-Hodgkin lymphomas.[1]
Etymology
It is named after the Swedish physician Jan G. Waldenström (1906-1996), who first identified the condition.
History and classification
WM was first described by Jan G. Waldenström (1906-1996) in 1944 in two patients with bleeding from the nose and mouth, anemia, decreased levels of fibrinogen in the blood (hypofibrinogenemia), swollen lymph nodes, neoplastic plasma cells in bone marrow, and increased viscosity of the blood due to increased levels of a class of heavy proteins called macroglobulins.[2]
For a period of time, WM was considered to be related to multiple myeloma due to the presence of monoclonal gammopathy and infiltration of the bone marrow and other organs by plasmacytoid lymphocytes. The new World Health Organization (WHO) classification, however, places WM under the category of lymphoplasmacytic lymphomas, itself a subcategory of the indolent (low-grade) non-Hodgkin lymphomas. [3]
Epidemiology
WM is a rare disorder, with fewer than 1,500 cases occurring in the United States annually.[1] The median age of onset of WM is between 60 and 65 years.[4][1]
Symptoms
Symptoms of WM include weakness, fatigue, weight loss and chronic oozing of blood from the nose and gums.[5] Peripheral neuropathy can occur in 10% of patients. Lymphadenopathy, splenomegaly, and/or hepatomegaly are present in 30-40% of cases.[4] Some symptoms are due to the effects of the IgM paraprotein, which may cause autoimmune phenomenon or cryoglobulinemia. Other symptoms of WM are due to the hyperviscosity syndrome, which is present in 6-20% of patients.[6][7][8][9] This is attributed to the IgM monoclonal protein increasing the viscosity of the blood. Symptoms of this are mainly neurologic and can include blurring or loss of vision, headache, and (rarely) stroke or coma.
Diagnosis
A distinguishing feature of WM is the presence of an IgM monoclonal protein (or paraprotein) that is produced by the cancer cells, and a concurrent decrease in levels of uninvolved immunoglobulins (i.e., IgG and IgA).
Laboratory Tests
- The laboratory diagnosis of Waldenström's macroglobulinemia is contingent on demonstrating a significant monoclonal IgM spike and identifying malignant cells consistent with Waldenström's macroglobulinemia (usually found in bone marrow biopsy samples and aspirates).
- General studies include a CBC, red cell indices, platelet count, and a peripheral smear.
- Normocytic normochromic anemia, leukopenia, and thrombocytopenia may be observed. Anemia is the most common finding, present in 80% of patients with symptomatic Waldenström's macroglobulinemia.
- The peripheral smear may reveal plasmacytoid lymphocytes, normocytic normochromic red cells, and rouleaux formation.
- Neutropenia can be found in some patients.
- Thrombocytopenia is found in approximately 50% of patients with bleeding diathesis.
- Chemistry tests include lactate dehydrogenase (LDH) levels, uric acid levels, erythrocyte sedimentation rate (ESR), renal and hepatic function tests, total protein levels, and an albumin-to-globulin ratio. The ESR and uric acid level may be elevated.
- Creatinine is occasionally elevated and electrolytes are occasionally abnormal. Hypercalcemia is noted in approximately 4% of patients.
- The LDH level is frequently elevated, indicating the extent of Waldenström's macroglobulinemia–related tissue involvement.
- Rheumatoid factor, cryoglobulins, direct antiglobulin test and cold agglutinin titre results can be positive.
- Beta-2-microglobulin and C-reactive protein test results are not specific for Waldenström's macroglobulinemia. Beta-2-microglobulin is elevated in proportion to tumor mass.
- Coagulation abnormalities may be present. Prothrombin time, activated partial thromboplastin time, thrombin time, and fibrinogen tests should be performed. Platelet aggregation studies are optional.
- Serum protein electrophoresis results indicate evidence of a monoclonal spike but cannot establish the spike as IgM. An M component with beta-to-gamma mobility is highly suggestive of Waldenström's macroglobulinemia.
- Immunoelectrophoresis and immunofixation studies help identify the type of immunoglobulin, the clonality of the light chain, and the monoclonality and quantitation of the paraprotein.
- High-resolution electrophoresis and serum and urine immunofixation are recommended to help identify and characterize the monoclonal IgM paraprotein.
- The light chain of the monoclonal protein is usually the kappa light chain. At times, patients with Waldenström's macroglobulinemia may exhibit more than one M protein.
- Plasma viscosity must be measured.
- Results from characterization studies of urinary immunoglobulins indicate that light chains (Bence Jones protein), usually of the kappa type, are found in the urine.
- Urine collections should be concentrated.
- Bence Jones proteinuria is observed in approximately 40% of patients and exceeds 1 g/d in approximately 3% of patients.
- Patients with findings of peripheral neuropathy should have nerve conduction studies and antimyelin associated glycoprotein serology
Prognosis
Current medical treatments result in survival some longer than 10 years. In part this is because better diagnostic testing means early diagnosis and treatments. Older diagnosis and treatments resulted in published reports of median survival of approximately 5 years from time of diagnosis.[1] New treatments have made longer term survival a reality for many with this condition. In rare instances, WM progresses to multiple myeloma.[10]
Treatment
There is no single accepted treatment for WM. Indeed, in 1991, Waldenström himself raised the question of the need for effective therapy.[11] In the absence of symptoms, many clinicians will recommend simply monitoring the patient.
In 2002, a panel at the International Workshop on Waldenström's Macroglobulinemia agreed on criteria for the initiation of therapy. They recommended starting therapy in patients with constitutional symptoms such as recurrent fever, night sweats, fatigue due to anemia, weight loss, progressive symptomatic lymphadenopathy or splenomegaly, and anemia due to bone marrow infiltration.
Complications such as hyperviscosity syndrome, symptomatic sensorimotor peripheral neuropathy, systemic amyloidosis, renal insufficiency, or symptomatic cryoglobulinemia were also suggested as indications for therapy.[12]
Treatment includes the monoclonal antibody rituximab, sometimes in combination with chemotherapeutic drugs such as chlorambucil, cyclophosphamide, or vincristine. Corticosteroids may also be used in combination. Plasmapheresis can be used to treat the hyperviscosity syndrome by removing the paraprotein from the blood, although it does not address the underlying disease.[13]
Recently, autologous bone marrow transplantation has been added to the available treatment options.[14][15][16][17]
References
- ↑ 1.0 1.1 1.2 1.3 Cheson BD (2006). "Chronic Lymphoid Leukemias and Plasma Cell Disorders", in Dale DD, Federman DD: ACP Medicine. New York, NY: WebMD Professional Publishing. ISBN 0974832715.
- ↑ Waldenstrom J (1944). "Incipient myelomatosis or "essential" hyperglobulinemia with fibrinognenopenia-a new syndrome?". Acta Med Scand 117: 216-247.
- ↑ Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD (2000). "The World Health Organization classification of neoplastic diseases of the haematopoietic and lymphoid tissues: Report of the Clinical Advisory Committee Meeting, Airlie House, Virginia, November 1997". Histopathology 36 (1): 69-86. PMID 10632755.
- ↑ 4.0 4.1 Raje N, Hideshima T, Anderson KC (2003). "Plasma Cell Tumors", in Kufe DW, Pollock RE, Weichselbaum RR, Bast RC, Gansler TS: Holland-Frei Cancer Medicine, 6th edition, New York, NY: B.C. Decker. ISBN 1550092138.
- ↑ Kyle RA (1998). "Chapter 94: Multiple Myeloma and the Dysproteinemias", in Stein JH: Internal Medicine, 5th ed., New York: C.V.Mosby. ISBN 0815186983.
- ↑ Owen RG, Barrans SL, Richards SJ, O'Connor SJ, Child JA, Parapia LA, Morgan GJ, Jack AS (2001). "Waldenstrom macroglobulinemia. Development of diagnostic criteria and identification of prognostic factors". Am J Clin Pathol 116 (3): 420-8. PMID 11554171.
- ↑ San Miguel JF, Vidriales MB, Ocio E, Mateo G, Sanchez-Guijo F, Sanchez ML, Escribano L, Barez A, Moro MJ, Hernandez J, Aguilera C, Cuello R, Garcia-Frade J, Lopez R, Portero J, Orfao A (2003). "Immunophenotypic analysis of Waldenstrom's macroglobulinemia". Semin Oncol 30 (2): 187-95. PMID 12720134.
- ↑ Ghobrial IM, Witzig TE (2004). "Waldenstrom macroglobulinemia". Curr Treat Options Oncol 5 (3): 239-47. PMID 15115652.
- ↑ Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP (2005). "Diagnosis and management of Waldenstrom's macroglobulinemia". J Clin Oncol 23 (7): 1564-77. PMID 15735132.
- ↑ Johansson B, Waldenstrom J, Hasselblom S, Mitelman F (1995). "Waldenstrom's macroglobulinemia with the AML/MDS-associated t(1;3)(p36;q21)". Leukemia 9 (7): 1136-8. PMID 7630185.
- ↑ Waldenstrom J (1991). "To treat or not to treat, this is the real question". Leuk Res 15 (6): 407-8. PMID 1907339.
- ↑ Kyle RA, Treon SP, Alexanian R, Barlogie B, Bjorkholm M, Dhodapkar M, Lister TA, Merlini G, Morel P, Stone M, Branagan AR, Leblond V (2003). "Prognostic markers and criteria to initiate therapy in Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia". Semin Oncol 30 (2): 116-20. PMID 12720119.
- ↑ Gertz MA (2005). "Waldenstrom macroglobulinemia: a review of therapy". Am J Hematol 79 (2): 147-57. PMID 15929102.
- ↑ Yang L, Wen B, Li H, Yang M, Jin Y, Yang S, Tao J (1999). "Autologous peripheral blood stem cell transplantation for Waldenstrom's macroglobulinemia". Bone Marrow Transplant 24 (8): 929-30. PMID 10516708.
- ↑ Martino R, Shah A, Romero P, Brunet S, Sierra J, Domingo-Albos A, Fruchtman S, Isola L (1999). "Allogeneic bone marrow transplantation for advanced Waldenstrom's macroglobulinemia". Bone Marrow Transplant 23 (7): 747-9. PMID 10218857.
- ↑ Anagnostopoulos A, Dimopoulos MA, Aleman A, Weber D, Alexanian R, Champlin R, Giralt S (2001). "High-dose chemotherapy followed by stem cell transplantation in patients with resistant Waldenstrom's macroglobulinemia". Bone Marrow Transplant 27 (10): 1027-9. PMID 11438816.
- ↑ Tournilhac O, Leblond V, Tabrizi R, Gressin R, Senecal D, Milpied N, Cazin B, Divine M, Dreyfus B, Cahn JY, Pignon B, Desablens B, Perrier JF, Bay JO, Travade P (2003). "Transplantation in Waldenstrom's macroglobulinemia--the French experience". Semin Oncol 30 (2): 291-6. PMID 12720155.
External links
- The International Waldenström's Macroglobulinemia Foundation site
- National Cancer Institute's Waldenström's Macroglobulinemia Q&A
- American Cancer Society Detailed Guide: Waldenström's Macroglobulinemia
- Research Fund for Waldenström's
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
