Updated ESMO guidelines on the management of marginal zone lymphomas


  • Dawn O'Shea
  • Univadis Medical News
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ESMO has published updated guidelines on the management of marginal zone lymphomas (MZL).

Key recommendations include the following:

Staging and risk assessment

  • Immunohistochemistry panel for all MZL subtypes including at least CD20, CD10, CD5, CD23, cyclin D1 and IgD.
  • Haemoglobin concentration, platelet count, LDH level and extrahilar lymphadenopathy (HPLL), Marginal Zone Lymphoma International Prognostic Index (MALT-IPIto estimate clinical behaviour.

Extranodal marginal zone B-cell lymphoma (EMZL)

  • Helicobacter pylori eradication therapy for gastric MZL.
  • Involved-site radiotherapy (ISRT) is preferred for localised EMZL.
  • Anti-HCV therapy for HCV-associated lymphoma.
  • Alternative treatments include chemotherapy (ChT), immunotherapy or combination chemoimmunotherapy.

Splenic marginal zone B-cell lymphoma (SMZL)

  • Rituximab alone is preferred initial therapy.
  • Chemoimmunotherapy is indicated when rituximab alone is ineffective or in the presence of disseminated symptomatic disease and/or signs of high-grade transformation.
  • Anti-HCV therapy for HCV-associated lymphoma.
  • Splenectomy in selected cases, when rituximab is not indicated/effective.

Nodal splenic marginal zone B-cell lymphoma (NMZL)

  • Treatment follows principles for follicular lymphoma.
  • Anti-HCV therapy for HCV-associated lymphoma.
  • Chemoimmunotherapy (e.g. R-bendamustine, R-CHOP, R-CVP).

Follow-up

  • Asymptomatic patients with disseminated MZL (SMZL and NMZL) on watch-and-wait should be assessed every six months
  • EMZL at non-gastric sites, follow-up every three months for first two years, and every six months thereafter.