Waldenström's macroglobulinemia
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For patient information on this page, click here Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2] Roukoz A. Karam, M.D.[3], Mirdula Sharma, MBBS [4]
Synonyms and keywords: Waldenstrom/Waldenstrom's macroglubulinemia (WM), Primary macroglobulinemia, Lymphoplasmacytic lymphoma (LPL), Plasmacytoid lymphocytic lymphoma, Familial Waldenstrom's Macroglobulinaemia, Waldenström macroglobulinaemia
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Waldenström's Macroglobulinemia from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic criteria of Waldenstrom Macroglobulinemia
In September 26-30, 2002, in Athens, Greece,the Second International Workshop was held in which the following diagnostic criteria for Waldenstrom's Macroglobulinemia was proposed:[1]
- IgM monoclonal protein of any concentration.
- Bone marrow infiltration by small lymphocytes showing plasmacytoid/plasma-cell differentiation.
- Intertrabecular pattern of bone marrow infiltration.
- Surface IgM+, CD5±, CD10−, CD19+, CD20+, CD22+, CD23−, CD25+, CD27+, FMC7+, CD103−, CD138 immunophenotype.
(3,4 are supportive of but not necessary for WM diagnosis).
- Another diagnostic Criteria for Waldenström Macroglobulinemia and Associated Disorders is as follow:[2]
- Waldenström macroglobulinemia:
- IgM monoclonal gammopathy (regardless of the size of the M protein) with >10% bone marrow lymphoplasmacytic infiltration (usually intertrabecular) by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation and a typical immunophenotype (surface IgM+, CD5–, CD10–, CD19+, CD20+, CD23–) that satisfactorily excludes other lymphoproliferative disorders, including chronic lymphocytic leukemia and mantle cell lymphoma.
- IgM MGUS:
- Serum IgM monoclonal protein level <3 g/dL, bone marrow lymphoplasmacytic infiltration <10%, and no evidence of anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly.
- Smoldering Waldenström macroglobulinemia (also referred to as indolent or asymptomatic Waldenström macroglobulinemia):
- Serum IgM monoclonal protein level ≥3 g/dL and/or bone marrow lymphoplasmacytic infiltration ≥10% and no evidence ofend-organ damage, such as anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly, that can be attributed to a lymphoplasmacytic proliferative disorder.
- Not all the diagnostic tests mentioned are performed in a WM patient. A doctor takes into account the following factors before choosing diagnostic tests in a particular patient:
- Suspected type of cancer.
- Signs and symptoms.
- Age.
- Medical condition of the patient.
- Results of earlier medical tests.
Diagnostic Study of Choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Electrocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies
Case Studies
- ↑ Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP (2005). "Diagnosis and management of Waldenstrom's macroglobulinemia". J Clin Oncol. 23 (7): 1564–77. doi:10.1200/JCO.2005.03.144. PMID 15735132.
- ↑ Ansell, Stephen M.; Kyle, Robert A.; Reeder, Craig B.; Fonseca, Rafael; Mikhael, Joseph R.; Morice, William G.; Bergsagel, P. Leif; Buadi, Francis K.; Colgan, Joseph P.; Dingli, David; Dispenzieri, Angela; Greipp, Philip R.; Habermann, Thomas M.; Hayman, Suzanne R.; Inwards, David J.; Johnston, Patrick B.; Kumar, Shaji K.; Lacy, Martha Q.; Lust, John A.; Markovic, Svetomir N.; Micallef, Ivana N.M.; Nowakowski, Grzegorz S.; Porrata, Luis F.; Roy, Vivek; Russell, Stephen J.; Short, Kristen E. Detweiler; Stewart, A. Keith; Thompson, Carrie A.; Witzig, Thomas E.; Zeldenrust, Steven R.; Dalton, Robert J.; Rajkumar, S. Vincent; Gertz, Morie A. (2010). "Diagnosis and Management of Waldenström Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines". Mayo Clinic Proceedings. 85 (9): 824–833. doi:10.4065/mcp.2010.0304. ISSN 0025-6196.