Search Database ICD-O-3 Code Lists

Name

Lymphoplasmacytic lymphoma (LPL)

ICD-O-3 Morphology

9671/3: Malignant lymphoma, lymphoplasmacytic
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes, bone marrow and other organs
Spleen involvement is common due to dissemination of disease

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Lymphoplasmacytic lymphoma (LPL) 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)

LPL typically involves the lymph nodes and bone marrow and frequently disseminates to the spleen. LPL cells may infrequently involve other extranodal sites, including the CNS (Bing–Neel syndrome), skin, pleural cavities (resulting in malignant effusions), or other sites usually involved by extranodal marginal zone lymphoma.

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: PH17

Alternate Names

Immunocytoma [OBS]
Malignant lymphoma, lymphoplasmacytoid

Definition

Lymphoplasmacytic lymphoma (LPL) is a neoplasm comprising small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving the bone marrow and sometimes involving the lymph nodes and spleen. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

ARIDA
IG gene arrangement
CXCR4 5338X
MYD88 L265P

Immunophenotyping

CD19+ (expression/positive)
CD20+ (expression/positive)
CD22+ (expression/positive)
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)
PAX5+ (expression/positive)

Treatments

Chemotherapy
Hormone 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)
C88.00 Waldenstrom macroglobulinemia, in remission (effective October 01, 2024)

Signs and Symptoms

Adenopathy
Anemia
Drenching night sweats
Fatigue
Fever (for no known reason)
Hepatomegaly
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Splenomegaly
Weakness
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
Peripheral blood smear
PET (positron emission tomography) scan

Progression and Transformation

Transformation to DLBCL occurs in a small proportion and indicates poor survival

Epidemiology and Mortality

Incidence: 3-7 cases per 1-million-person years
Age: age 65 and older
Sex: male predilection
Survival: 5-10 years median survival

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: Lymphoplasmacytic Lymphoma
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: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Indolent B-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/18/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/indolent-b-cell-lymphoma-treatment-pdq. Accessed <03/04/2025>. [PMID: 37437080]
Section: Indolent B-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary