Lymphoplasmacytic lymphoma

Name
Lymphoplasmacytic lymphoma
ICD-O-1 Morphology
9611/3
Effective 1978 - 1991
ICD-O-2 Morphology
9671/3
Effective 1992 - 2000
ICD-O-3 Morphology
9671/3
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

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Grade
6 - B-cell
Module Rule
Module 6: PH17
Alternate Names
Immunocytoma [OBS]
LPL
Malignant lymphoma, lymphoplasmacytic
Malignant lymphoma, lymphoplasmacytoid
Malignant lymphoma, plasmacytoid [OBS]
Plasmacytic lymphoma [OBS]
Definition
Neoplasm of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow, sometimes lymph nodes, spleen; usually lacking CD5; serum monoclonal protein of IgM in most cases with consequent hyperviscosity symptoms.
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
IG gene arrangement
Immunophenotyping
IgA positive (rare)
IgG positive
IgM monclonal gammopathy positive
Treatments
Chemotherapy
Hormone
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 (effective October 1, 2015 U.S. only)
C83.0 Small cell B-cell lymphoma
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
Bone marrow aspiration and biopsy
CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
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
Survival: 5-10 years median survival