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Marginal zone lymphoma

Key sources
The following summarized guidelines for the evaluation and management of marginal zone lymphoma are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2023) and the European Society of Medical Oncology (ESMO 2020).


1.Screening and diagnosis

As per BSH 2023 guidelines:
Diagnose nodal and extranodal MZL based on histomorphology and immunohistochemical markers.
Diagnose splenic MZL based on a combination of morphology, flow cytometry on peripheral blood, and marrow aspirate/trephine biopsy histology/immunohistochemistry.
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2.Classification and risk stratification

Prognosis: use clinical and biological prognostic systems (HPLL, MALT-IPI) in clinical routine to estimate clinical behavior.

3.Diagnostic investigations

Initial evaluation: elicit history, perform a physical examination, and obtain the following laboratory tests in the initial staging of all MZL subtypes:
CBC and differential
liver function tests
renal function tests
protein electrophoresis
β-2 microglobulin
serum and urine immunofixation
serology for HBV, HCV, HIV, and cryoglobulins and cryocrit if HCV-positive

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  • Imaging for staging

4.Diagnostic procedures

Biopsy and histopathology: as per BSH 2023 guidelines, perform bone marrow biopsy for staging of nodal or splenic MZL and in cases of non-gastric extranodal MZL/MALT to investigate cytopenias or confirm localized disease where radiotherapy is planned.
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  • Upper gastrointestinal endoscopy

5.Medical management

Management of clonal B-cell lymphocytosis of marginal zone origin
Recognize that clonal B-cell lymphocytosis of marginal zone origin is usually indolent and should not be viewed as a manifestation of lymphoma unless progression occurs.
Consider obtaining annual monitoring for symptoms and signs of splenomegaly and progressive cytopenias after specialist assessment and appropriate patient education.

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  • Management of nodal MZL

  • Management of splenic MZL (watchful waiting)

  • Management of splenic MZL (antiviral therapy)

  • Management of splenic MZL (chemoimmunotherapy)

  • Management of splenic MZL (splenectomy/splenic radiotherapy)

  • Management of extranodal MZL (gastric, localized)

  • Management of extranodal MZL (gastric, advanced)

  • Management of extranodal MZL (non-gastric)

  • Management of extranodal MZL (CNS)

  • Management of histological transformation

6.Follow-up and surveillance

Management of relapse: consider obtaining active monitoring in asymptomatic patients with relapsed disease (any stage).
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