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Marginal Zone Lymphoma Case Studies

First-Line Therapy in Advanced MZL

Ariela Noy, MD
Published Online:Aug 27, 2018
Ariela Noy, MD, discusses the rationale for treating a 64-year-old woman with advanced extranodal marginal zone lymphoma (MZL) with ibrutinib after rituximab therapy and disease progression.

Second-Line Therapy in Extranodal Marginal Zone Lymphoma


Ariela Noy, MD: When a physician makes a decision to treat a patient with first-line therapy, multiple factors come into play. Most commonly, patients are treated with either immunotherapy alone—currently, rituximab is the only antibody approved for that indication—or a combination of immunotherapy and chemotherapy. Similar to follicular lymphoma, this decision is somewhat subjective. Most commonly, the expectation is that patients with relatively low-bulk disease will do well with immunotherapy alone and the relatively rare patients with bulky disease need combined immunotherapy.

Unlike follicular lymphoma, we really have a dearth of randomized first-line trials. Sometimes that decision is, perhaps, more subjective and less scientific than we would like, but this is what we have in terms of data.

Looking at rituximab/bendamustine versus R-CHOP, we do have a randomized trial, though it wasn’t dedicated specifically to marginal zone lymphoma patients. That trial, the StiL trial, predominantly compared follicular lymphoma patients with stage III and IV disease and included other subtypes. The marginal zone lymphoma patients were really a small cohort. We can infer, from the larger results of the trial, however, that rituximab/bendamustine compared favorably with R-CHOP. In addition, the side effect profile of R-CHOP far outstrips, negatively, the rituximab-bendamustine regimen.

There’s a second trial, which is a little problematic, called The BRIGHT trial. That trial raised some concerns about the toxicity profile of rituximab/bendamustine. However, most medical oncologists currently prefer rituximab/bendamustine over R-CHOP.

The options for follicular lymphoma, compared with the options for marginal zone lymphoma as first-line therapy, are currently similar. What we don’t know, for example, is whether other drugs that may currently be used as second-line therapy—for example, ibrutinib—would compare favorably in the first-line setting. And so, this is a trial that we hope to begin this year.

Transcript edited for clarity.

A 64-Year-Old Woman With Advanced Extranodal MZL

January 2016

  • PH: At age 64, the patient presented with a fever of unknown origin, weight loss, and fatigue
    • PE: revealed 2 masses near left ear
    • PMH: Sjogren’s syndrome, symptoms managed on cevimeline
  • CT revealed bilateral involvement in parotid glands and a 3.0-cm. mass in the left lung
  • Biopsies confirmed presence of MALT lymphoma in salivary gland and lung with nodules of diffuse heterogeneous B-cell infiltrate
  • IHC: B cell phenotype CD20
  • HBC, HBV, and other infections ruled out

Treatment History

  • After 6-month period of active monitoring/observation, salivary masses began to cause patient distress; she also developed a persistent cough
    • CT revealed an additional new mass in left lung
  • Decision was made to start patient on a course of rituximab
  • Follow-up imaging at 6 months and 9 months showed near complete remission

March 2018

  • Imaging at 20 months showed disease progression in the lung  
  • Patient started on treatment with rituximab monotherapy

June 2018

  • Imaging at 3 months showed no response to therapy
  • The patient was started on treatment with ibrutinib
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