Name

Lymphoplasmacytic lymphoma

ICD-O-1 Morphology

9611/3: Malignant lymphoma, lymphoplasmacytoid type
Effective 1978 - 1991

ICD-O-2 Morphology

9671/3: Malignant lymphoma, lymphoplasmacytic
Effective 1992 - 2000

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 6
Most common sites of involvement: lymph nodes, bone marrow and other organs

Grade

Not Applicable

Module Rule

Module 6: PH17

Alternate Names

Immunocytoma [OBS]
LPL
Malignant lymphoma, lymphoplasmacytic
Malignant lymphoma, lymphoplasmacytoid

Definition

Lymphoplasmacytic lymphoma (LPL) is a neoplasm of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow and sometimes lymph nodes and spleen, which does not fulfill the criteria for any of the other small B-cell lymphoid neoplasms that can also have plasmacytic differentiation.

Abstractor Notes

LPL is an NOS which has two variants. All three diseases are diagnosed by an increased number of immunoglobulins. WM is caused by increased lymphocytes which causes an increase in IgM. Gamma heavy chain disease is caused by increased plasma cells which results in an increase of IgG. LPL has mixed abnormalities, both the lymphocytes and plasma cells are increased which results in an abnormally high IgM AND IgG.

1. Waldenstrom Macroglobulinemia (WM) (9761/3) is a subset of LPL. WM is caused by increased lymphocytes which causes an increase in IgM. See the abstractor notes for WM for more information.

2. Gamma heavy chain disease (9762/3) is a variant of LPL. Gamma heavy chain disease is caused by increased plasma cells which results in an increase of IgG. See the abstractor notes for Gamma heavy chain disease for more information.

All three diseases are diagnosed by an increased number of immunoglobulins.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Histologic confirmation
Immunophenotyping
Peripheral blood smear

Genetics Data

ARIDA
IG gene arrangement
CXCR4 5338X
MYD88 L265P

Immunophenotyping

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

Treatments

Chemotherapy
Hormone therapy

Corresponding ICD-9 Codes

200.8 Other named variants, lymphoma

Corresponding ICD-10 Codes

C83.0 Diffuse non-Hodgkin's disease, small cell (diffuse)

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

C83.0 Small cell B-cell lymphoma (effective October 01, 2015)

Signs and Symptoms

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

Diagnostic Exams

Progression and Transformation

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

Epidemiology and Mortality

Age: 60 years median age
Sex: male predominance

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 Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary