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
Primary site must be blood (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. 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.

Treatment of Waldenstrom macroglobulinemia may include the following:
1. Biologic (immuno) therapy with interferon
2. Chemotherapy
3. Plasmapheresis
4. Proteasome inhibitor
5. Watchful waiting
Definitive Diagnostic Methods
Genetic testing
Immunophenotyping
Serum paraprotein
Genetics Data
6q deletion in 50% of cases
Somatic hypermutations in V regions of IG genes (IgM), t(9;14)(p13;q32) 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
Watchful waiting (for asymptomatic patients)
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
Easy bruising or bleeding (nosebleeds or bleeding from gums)
Elevated lymphocytes in blood
Feeling very tired
Headaches
Pain or a feeling of fullness below the ribs on the left side
Pain, tingling, or numbness, especially in the hands, feet, fingers, or toes
Painless lumps in the neck, underarm, stomach, or groin
Vision changes, such as blurred vision or blind spots
Weight loss for no reason
Progression and Transformation
None
Epidemiology and Mortality
None