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Marginal Zone Lymphoma Case Studies

Ibrutinib As Second-Line Therapy in Refractory 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: The open-label trial that garnered FDA approval of ibrutinib in the second-line setting included patients who had used rituximab or rituximab/chemotherapy as first-line treatment. The drug works in marginal zone lymphoma using a mechanism of action of inhibition of the protein Bruton’s tyrosine kinase.

Interestingly, a similar trial in follicular lymphoma patients was not successful. Consequently, patients with marginal zone lymphoma have a second or beyond treatment option that follicular lymphoma patients do not have. The patients who receive ibrutinib can receive treatment indefinitely. At this time, the duration of therapy is not known.

Patients who are treated with ibrutinib as second-line therapy typically have a good survival outcome. Currently, we don’t really know what that is. We only have data for about a median of 2 years. However, at least two-thirds of the patients remain without additional therapy and about 80% of the patients are alive after 3 years of follow-up.

One of the important questions that we expect to explore over the next couple of years is whether it can be used in the first-line setting. Specifically, we are about to launch a trial looking at ibrutinib in combination with rituximab. Some patients will receive rituximab alone and the others will receive rituximab and ibrutinib.

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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