Search Database ICD-O-3 Code Lists

Name

Waldenstrom macroglobulinemia (WM)

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) for cases diagnosed 1/1/2018 and forward. For cases diagnosed 2010-2017, primary site must be blood (C420).

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Waldenstrom macroglobulinemia (WM) is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)

If the diagnosis states "Lymphoplasmacytic Lymphoma (LPL)/Waldenstrom's macroglobulinemia (WM)" use the following criteria to determine the appropriate histology code.

Code to WM (9761/3) when
1. There is a confirmed diagnosis of LPL in the bone marrow AND a clinical diagnosis of WM
2. Clinical diagnosis of WM

Code to LPL (9671/3) when
1. There is an absence of IgM in the blood or it's unknown if IgM is in the blood OR
2. Bone marrow is not involved with LPL or it's unknown if the bone marrow is involved with LPL

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

Module 6: PH16

Alternate Names

IgM LPL/ Waldenstrom macroglobulinemia type
IgM-type lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia (IGM-type LPL/WM)
Non-IgM LPL/ Waldenstrom macroglobulinemia type
Non-IgM-type lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia (IGM-type LPL/WM)

Definition

Waldenström macroglobulinemia (WM) is defined by the combination of LPL in the bone marrow and an IgM monoclonal component in the blood. (WHO 5th edition)

Definitive Diagnostic Methods

Clinical diagnosis
Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

ARIDA
IG gene rearrangement
CXCR4 5338X
MYD88 L265P

Immunophenotyping

CD5- (no expression/negative)
CD10- (no expression/negative)
CD19+ (expression/positive)
CD20+ (expression/positive)
CD22+ (expression/positive)
CD23- (no expression/negative)
CD25+ (expression/positive)
CD38+ (expression/positive)
CD45+ (expression/positive)
CD79a+ (expression/positive)
CD103(no expression/negative)
CD138+ (expression/positive)
IgA+ (expression/positive) (rare)
IgD- (no expression/negative)
IgG+ (expression/positive)
IgM monclonal gammopathy+ (expression/positive)

Treatments

Chemotherapy
Hormone therapy
Immunotherapy
Other therapy

Transformations to

Transformations from

None

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C88.0 Waldenstrom macroglobulinemia

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

C88.0 Waldenstrom macroglobulinemia (effective October 01, 2015 - September 30, 2024)
C88.00 Waldenstrom macroglobulinemia, not having achieved remission (effective October 01, 2024)
C88.01 Waldenstrom macroglobulinemia, in remission (effective October 01, 2024)

Signs and Symptoms

Adenopathy
Confusion
Dizziness
Easy bruising or bleeding (nosebleeds or bleeding from gums)
Elevated lymphocytes in blood
Feeling very tired
Headaches
Hepatomegaly
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
Splenomegaly
Vision changes, such as blurred vision or blind spots
Weight loss for no reason

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Peripheral blood smear

Progression and Transformation

None

Epidemiology and Mortality

None

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mature B-cell neoplasms
Pages: Part B: 394-398

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: Non-IgM monoclonal gammopathy of undetermined significance
Glossary