Splenic marginal zone lymphoma

Name
Splenic marginal zone lymphoma
ICD-O-3 Morphology
9689/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C422

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Grade
6 - B-cell
Module Rule
None
Alternate Names
SLVL
SMZL
Splenic B-cell marginal zone lymphoma
Splenic lymphoma with circulating villous lymphocytes
Splenic lymphoma with villous lymphocytes
Splenic marginal zone B-cell lymphoma
Splenic marginal zone lymphoma, NOS
Splenic MZL
Definition
Very rare disease characterized by lymphoid infiltrate in splenic white pulp growing in nodular pattern replacing pre-existing follicles with variable degree of red pulp infiltration; small lymphocytes predominant in central areas, medium-sized cells present in periphery. 66% present with circulating villous lymphocytes.
Abstractor Notes
Splenomegaly is seen in almost all patients, commonly without lymphadenopathy. The bone marrow is frequently positive. Nodal and extranodal involvment are rare.

The lack of CD5 expression is helpful in the discrimination between SMZL and chronic lymphocytic leukemia/small lymphocytic lymphoma, and the lack of CD10 expression argues against follicular lymphoma. Mantle cell lymphoma is excluded due to the lack of CD5 and cyclin-D1 expression.
Definitive Diagnostic Methods
Flow cytometry
Histological confirmation
Genetics Data
Acute loss of chromosome 7q31-32
Immunoglobulin heavy and light chain rearrangement
Immunophenotyping
Annexin A1-(1014, 1428) neg
CD5-
CD10-
CD20+
CD23-
CD43-
CD79a+
CD103-
Cyclin D1 absent
Dysregulation of CD6K gene at 7q21
Surface IgD
Surface IgM
Treatments
Chemotherapy
Immunotherapy
Surgery
Transformations from
None
Corresponding ICD-9 Codes
200.3 Marginal zone 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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None