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Splenic marginal zone lymphoma
Other Resources UpToDate PubMed

Splenic marginal zone lymphoma

Contributors: Nina Haghi MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

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

Codes

ICD10CM:
C83.07 – Small cell B-cell lymphoma, spleen

SNOMEDCT:
128802003 – Splenic marginal zone B-cell lymphoma

Look For

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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).

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:08/26/2018
Last Updated:09/05/2018
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Splenic marginal zone lymphoma
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A medical illustration showing key findings of Splenic marginal zone lymphoma : Fatigue, Lymphocytosis, Splenomegaly, Abdominal discomfort
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