Treating Marginal Zone Lymphoma (MZL)

Indicators for Treatment and Select First Line Therapy Options for MZL

The GELF Criteria were established in follicular lymphoma to help define patients with high tumor burden who are in need of treatment.1,2 These GELF criteria are also used frequently to identify patients with MZL who are in need of treatment, as opposed to those who may be managed with observation (also called “watchful waiting”).1

GELF Criteria

Indicators for Treatment1,2

Patients must meet ≥1 criteria to be considered "high" tumor burden:

  • Any tumor mass > 7 cm
  • 3 nodes in 3 distinct areas each > 3 cm
  • Any B symptom
  • Splenomegaly
  • Organ compression, pleural effusion, or ascites
  • Leukemia (>5.0 x 109/L maligant cells) or cytopenia (leukocytes < 1.0 x 109/L and/or platelets < 100 x 109/L)
  • Elevated serum LDH or β2-microglobulin

Observation (Watchful Waiting)

Watchful waiting is typically used when lymphoma is slow-growing or disease is asymptomatic.3 During observation, patients receive periodic physical exams, lab tests, and imaging, with an emphasis on reportable symptoms.1,4

In MZL, observation may be recommended if re-evaluation suggests slowly responding disease or asymptomatic nonprogression, for patients whose diagnostic biopsy was excisional, or where RT could result in significant morbidity, or where the potential toxicity of ISRT outweighs potential clinical benefit.1 Treatment following observation is indicated when the patient experiences progressive disease or transformation.1

Follow-Up and Subsequent Therapy

The follow-up for MZL is similar to that of FL1:

Note: Patients with gastric MALT lymphoma should undergo re-staging and follow-up endoscopy 3-6 months after ISRT or immunotherapy.1

Relapsed/refractory MZL

  • Treatment options for relapsed/refractory MZL are similar to second-line and subsequent treatment options for FL.1
  • Local relapse of MZL may be treated with ISRT. Relapsed disease that is asymptomatic and lacks indicators for treatment may be observed.1
  • Systemic treatment options for relapsed/refractory MZL include chemoimmunotherapy, targeted therapies, radioimmunotherapy, and clinical trials.1
  • Hematopoietic stem cell transplant (HSCT) may also be a treatment option for highly selected patients with relapsed/refractory MZL.1,5

Transformed MZL

  • Histologic transformation of MZL to an aggressive NHL can occur in all subtypes and is generally associated with a worsened prognosis.1
  • Selection of treatment for patients with transformed MZL must be highly individualized taking into account prior treatment history.1
  • Other treatment options for transformed MZL include radioimmunotherapy, ISRT, high-dose therapy with autologous stem cell rescue (HDT/ASCR) or allogeneic HSCT, and best supportive care.1

FL, follicular lymphoma; H&P, history and physical; HCV, hepatitis C virus; ISRT, involved site radiation therapy; MALT, mucosa-associated lymphoid tissue; RT, radiation therapy.

References: 1. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for B-Cell Lymphomas V.4.2019. © National Comprehensive Cancer Network, Inc. 2019. All rights reserved. Accessed July 10, 2019. To view the most recent and complete version of the guideline, go online to NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way. 2. Sebban C, Mounier N, Brousse N, et al. Standard chemotherapy with interferon compared with CHOP followed by high-dose therapy with autologous stem cell transplantation in untreated patients with advanced follicular lymphoma: the GELF-94 randomized study from the Groupe d'Etude des Lymphomes de l'Adulte (GELA). Blood. 2006;108:2540-2544. 3. Armitage JO, Longo DL. Is watch and wait still acceptable for patients with low-grade follicular lymphoma? Blood. 2016;127(23):2804-2808. 4. Leukaemia Foundation. "Watch and wait" management of indolent non-Hodgkin lymphomas. Accessed January 5, 2019. 5. Delinger NM et al. Management of relapsed/refractory marginal zone lymphoma: focus on ibrutinib. Cancer Management Res. 2018:10:615-624.