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

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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 IE, IIE1, IIE2, IIIE 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 ≥ IIE2 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.
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Keywords: intestinal; marginal zone B-cell lymphoma of MALT type

Document Type: Research Article

Affiliations: 1: Department of Internal Medicine, Dong-A University College of Medicine 2: Division of Hematology/Oncology, Department of Medicine 3: Department of Pathology, Samsung Medical Center 4: Department of Hematology and Oncology, Korea Institute of Radiological & Medical Sciences 5: Department of Internal Medicine, Gachon Medical School 6: Department of Internal Medicine, Hallym University College of Medicine, Busan, Korea

Publication date: October 1, 2007

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