Splenic marginal zone lymphoma
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Synopsis
SMZL is a very rare lymphoid neoplasm that occurs primarily in adults older than 50 years (median age is 67-68 years) with no sex predominance.
Patients present with splenomegaly and/or peripheral blood lymphocytes that appear villous. Thrombocytopenia and/or anemia may also be present. Patients are often asymptomatic at time of diagnosis, and initial abnormalities are identified incidentally on blood work.
An association between SMZL and chronic hepatitis C virus (HCV) has been identified in several case series, and in some cases, antiviral therapy for chronic HCV has resulted in regression of SMZL.
Related topics: Nodal marginal zone lymphoma; Extranodal marginal zone lymphoma
Codes
ICD10CM:C83.07 – Small cell B-cell lymphoma, spleen
SNOMEDCT:
128802003 – Splenic marginal zone B-cell lymphoma
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Hairy cell leukemia
- Mantle cell lymphoma
- Lymphoplasmacytic lymphoma
- Follicular lymphoma
- Splenomegaly can occur with other hematologic disorders including leukemia, other myeloproliferative neoplasms, and nonmalignant causes (autoimmune or infection).
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Last Reviewed:08/26/2018
Last Updated:09/05/2018
Last Updated:09/05/2018