Waldenstrom macroglobulinemia

Name
Waldenstrom macroglobulinemia
ICD-O-2 Morphology
9761/3
Effective 1992 - 2000
ICD-O-3 Morphology
9761/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
C420

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Grade
6 - B-cell
Module Rule
Module 6: PH16
Alternate Names
WM
Definition
Myeloproliferative disorder of B-lymphocytes producing abnormal plasma cells that secrete excess IgM causing hyperviscosity (thickening) of blood. WM is defined as a lymphoplasmacytic lymphoma (9671/3) with bone marrow involvement and IgM of any concentration. A significant subset of lymphoplasmacytic lymphoma patients has WM.

Neoplasm of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, involving bone marrow, lymph nodes, spleen; usually lacking CD5; serum monoclonal protein of IgM with consequent hyperviscosity symptoms.
Abstractor Notes
Waldenstrom Macroglobulinemia (WM) is a subset of lymphoplasmacytic lymphoma. Patients with WM have IgM (immunoglobulin M) in their blood and/or bone marrow. IgM is also called IgM monoclonal gammopathy. 15-30% of WM patients have splenomegaly, hepatomegaly, and/or adenopathy. There will also be an increased number of lymphocytes in the blood. A familial disposition may exist in up to 20% of patients. It tends to be diagnosed at a younger age than other lymphoplasmacytic lymphomas.
Definitive Diagnostic Methods
Serum paraprotein
Genetics Data
Somatic hypermutations in V regions of IG genes (IgM), t(9;14)(p13;q32) in 50% of cases
6q deletion in 50% of cases
Trisomy 4 in 20% of cases
WM has a homogenous gene expression profile other than 6q deletion, more similar to CLL and normal B-cells than to myeloma
Immunophenotyping
CD5-
CD10-
CD19+
CD20+
CD22+
CD23-
CD38+
CD79a+
CD103-
Surface/cytoplasmic IgM, typically IgD-
Treatments
Chemotherapy
Hormone
Immunotherapy
Transformations from
None
Corresponding ICD-9 Codes
273.3 Macroglobulinemia
Corresponding ICD-10 Codes
C88.0 Waldenstrom's macroglobulinemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C88.0 Waldenstrom's macroglobulinemia
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None