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Nodal marginal zone lymphoma
Other Resources UpToDate PubMed

Nodal marginal zone lymphoma

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

Synopsis

Nodal marginal zone lymphoma (NMZL) is a mature postgerminal center low-grade B-cell lymphoma that presents most commonly in peripheral lymph nodes. Peripheral blood and bone marrow involvement can also occur.

NMZL constitutes less than 2% of lymphoid neoplasms and most often occurs in adults with a median age of approximately 60 years old with no sex predominance. Patients with chronic hepatitis C infection are at increased risk. Pediatric nodal marginal zone lymphoma is a distinct entity with marked male predominance and localized disease.

Patients present with local or generalized lymphadenopathy B symptoms (such as fever, weight loss, or night sweats), or they may present with no symptoms at all. Approximately one-third of patients have concomitant extranodal marginal zone lymphoma; therefore, adequate surveillance for extranodal involvement should be performed, especially in patients with history of autoimmune disorders.

Related topics: Extranodal marginal zone lymphoma; Splenic marginal zone lymphoma

Codes

ICD10CM:
C83.00 – Small cell B-cell lymphoma, unspecified site

SNOMEDCT:
726721002 – Nodal marginal zone B-cell lymphoma

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Other B-cell lymphomas – Extranodal marginal zone B-cell lymphoma, Splenic marginal zone lymphoma, Mantle cell lymphoma, Chronic lymphocytic leukemia, Follicular lymphoma
  • Lymphoplasmacytic lymphoma
  • Multiple myeloma
  • Infection
  • Splenomegaly can occur with other hematologic disorders including leukemia and other myeloproliferative neoplasms, as well as from 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:08/07/2018
Last Updated:12/18/2018
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Nodal marginal zone lymphoma
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A medical illustration showing key findings of Nodal marginal zone lymphoma : Fever, Night sweats
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