Intestinal marginal zone B-cell lymphoma of MALT type: clinical manifestation and outcome of a rare disease

Eur J Haematol. 2007 Oct;79(4):287-91. doi: 10.1111/j.1600-0609.2007.00925.x. Epub 2007 Aug 10.

Abstract

Intestinal marginal zone B-cell lymphoma of the MALT type (I-MZL) is a relatively uncommon form of lymphoma. Twenty-seven patients with histologically-confirmed I-MZL were analyzed. The patients initially presented with abdominal pain (62.9%), and diarrhea (22.2%). The most common involved site was the ileo-caecal area (40.7%). Musshoff's stage I(E), II(E)1, II(E)2, III(E) and IV were present in 44%, 15%, 11%, 7.4% and 22% respectively. Sixty-three percent were in the low-risk group according to the Follicular Lymphoma International Prognostic Index. Complete response and partial response were achieved in 82% and 4% patients. The estimated 5-year overall survival (OS) and progression-free survival (PFS) rates were 86% and 54%. Stage > or = II(E)2 was determined to be a poor prognostic factor for PFS and OS. I-MZL commonly manifests in an early-stage, low-risk state and tends to respond well to local and systemic treatment with favorable prognosis. I-MZL tends to be an indolent disease - characterized by prolonged survival with frequent relapses, similarly to other site MZLs.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Cecal Neoplasms / diagnosis
  • Cecal Neoplasms / mortality*
  • Cecal Neoplasms / pathology
  • Cecal Neoplasms / therapy
  • Combined Modality Therapy
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Humans
  • Ileal Neoplasms / diagnosis
  • Ileal Neoplasms / mortality*
  • Ileal Neoplasms / pathology
  • Ileal Neoplasms / therapy
  • Lymphoma, B-Cell, Marginal Zone / diagnosis
  • Lymphoma, B-Cell, Marginal Zone / mortality*
  • Lymphoma, B-Cell, Marginal Zone / pathology
  • Lymphoma, B-Cell, Marginal Zone / therapy
  • Male
  • Middle Aged
  • Neoplasm Staging
  • Rare Diseases / diagnosis
  • Rare Diseases / mortality*
  • Rare Diseases / pathology
  • Rare Diseases / therapy
  • Recurrence
  • Risk Factors
  • Survival Rate