Splenic marginal zone lymphoma (SMZL) is an indolent low-grade B-cell lymphoma composed of small to intermediate-sized lymphocytes that proliferate within the spleen and splenic hilar lymph nodes. Bone marrow and peripheral blood are usually involved, but lymphadenopathy and extranodal disease are uncommon.
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
Splenic marginal zone lymphoma
Synopsis
Codes
ICD10CM:
C83.07 – Small cell B-cell lymphoma, spleen
SNOMEDCT:
128802003 – Splenic marginal zone B-cell lymphoma
C83.07 – Small cell B-cell lymphoma, spleen
SNOMEDCT:
128802003 – Splenic marginal zone B-cell lymphoma
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
To perform a comparison, select diagnoses from the classic differential
Subscription Required
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Reviewed:08/26/2018
Last Updated:09/05/2018
Last Updated:09/05/2018