What is marginal zone B-cell lymphoma (MZL)?

Updated: Aug 31, 2020
  • Author: Mark P Eid, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Answer

Marginal zone B-cell lymphoma (MZL) (MALT-type) is an indolent cutaneous lymphoma, accounting for approximately 10% of all cutaneous lymphomas. Variants include immunocytoma and primary cutaneous plasmacytoma. Although it has been reported in children, it is most commonly seen in patients in their fourth decade. [19, 20] The prognosis of MZL (MALT-type) is excellent, with a 5-year survival rate of greater than 95%.

Synonyms based on classification are as follows:

  • WHO classification (2008) - Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)

  • WHO/EORTC classification (2005) - Primary cutaneous MZL

  • WHO classification (2001) - Extranodal marginal zone lymphoma of MALT type

  • Revised European American Lymphoma (REAL) classification (1997) - Extranodal MZL

  • EORTC classification (1997) - Primary cutaneous MZL

Extranodal low-grade B-cell lymphoma of the MALT type and FCL are the most frequent types of peripheral B-cell neoplasms seen primarily in the skin. [21, 22, 23] Extranodal MZL may develop from reactive infiltrates that represent immune responses to external factors or autoantigens. [24] An etiopathological relationship to B burgdorferi has been demonstrated in some cases. [25, 26]


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