Name

Waldenstrom macroglobulinemia

ICD-O-2 Morphology

9761/3: Waldenstrom macroglobulinemia
Effective 1992 - 2000

ICD-O-3 Morphology

9761/3: Waldenstrom macroglobulinemia
Effective 2001 and later

Reportable

for cases diagnosed 1992 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421). Previously the default primary site was blood (C420).

Grade

Not Applicable

Module Rule

Module 6: PH16

Alternate Names

IgM Monoclonal gammopathy (see abstractor notes)
WM

Definition

Waldenstrom macroglobulinemia (WM) is found in a substantial subset of patients with LPL, but is not synonymous with it; it is defined as LPL with bone marrow involvement and an IgM monoclonal gammopathy of any concentration.

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

ARIDA
IG gene rearrangement
CXCR4 5338X
MYD88 L265P

Immunophenotyping

CD5-
CD10-
CD19 expressed
CD20 expressed
CD22 expressed
CD23-
CD25 expressed
CD38 expressed
CD45+
CD79a+
CD103-
CD138+
IgA positive (rare)
IgD negative
IgG positive
IgM monclonal gammopathy positive

Treatments

Chemotherapy
Hormone therapy
Immunotherapy
Other therapy

Transformations from

None

Corresponding ICD-9 Codes

273.3 Macroglobulinemia

Corresponding ICD-10 Codes

C88.0 Waldenstrom's macroglobulinemia

Corresponding ICD-10-CM Codes (U.S. only)

C88.0 Waldenstrom's macroglobulinemia (effective October 01, 2015)

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

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 232-235

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Glossary