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Marginal Zone Lymphoma Case Studies

Second-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: After a patient with disseminated disease has a relapse and the decision is made to retreat that patient, the physician has multiple options. Depending on the duration of the first remission, retreatment with rituximab may be a reasonable option, especially if the patient had a long disease-free interval and/or the patient is frail and other therapies may be too toxic. Patients who have a disease-free interval of under 1 year essentially should not be retreated with rituximab. Those patients are not likely to have a second response. If they do, it’s unlikely that it would be maintained.

The considerations for second-line therapy are similar but differ somewhat from the considerations in the first-line setting. The physician has many things to choose from, in terms of second-line therapy with chemoimmunotherapy. The patient could receive rituximab/bendamustine, R-CVP, R-CHOP, or, possibly, fludarabine-based therapy.

As of 2017, ibrutinib is also an FDA-approved option. Ibrutinib is a continuous oral therapy. Unlike chemotherapy, it has a better side effect profile. The treatment benefits about 88% of patients if one takes into consideration complete responses, partial responses, and disease shrinkage. The vast majority of patients will not have a complete response, however, with ibrutinib therapy. Currently, they maintain treatment with once-a-day oral dosing until they have disease progression or an adverse event that requires them to come off the drug or they simply don’t want to take pills any more.

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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