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Marginal Zone Lymphoma Case Studies

Advanced MZL: First-Line Treatment Considerations

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 decision to treat somebody with disseminated marginal zone lymphoma is similar to the decision to treat other indolent lymphomas. We have to keep in mind that these patients have long life expectancies. We do not want to overtreat patients, especially if the decision is made to treat them with drugs that have significant side effects. Even immunotherapy can cause side effects such as prolonged B-cell hypogammaglobulinemia, and this can leave a patient open to infection. When we add things like alkylating agents there is an added risk for a secondary malignancy such as acute myeloid leukemia or myeloproliferative neoplasia. As a result, we need to be judicious and have frank discussions with patients regarding if much their symptoms are bothering them and whether or not their case warrants therapy at this time.

We look at a number of different factors when we choose first-line therapy for patients with disseminated marginal zone lymphoma. One of the important things is the bulk of disease. The expectation is that patients with significantly bulky disease, which is a relatively rare occurrence, admittedly probably need more than immunotherapy, alone. The reason for that is you will more likely get a partial response with immunotherapy alone than with immunochemotherapy, which is associated with a high complete response rate. If you have a patient who has a high complete response rate, or one with a lot of tumor burden, single-agent rituximab will leave you with a relatively large disease burden if you only get a partial response.

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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