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Indolent small intestinal CD4+ T-cell lymphoma is a distinct entity with unique biologic and clinical features.

ABSTRACT: Enteropathy-associated T-cell lymphomas (EATL) are rare and generally aggressive types of peripheral T-cell lymphomas. Rare cases of primary, small intestinal CD4+ T-cell lymphomas with indolent behavior have been described, but are not well characterized. We describe morphologic, phenotypic, genomic and clinical features of 3 cases of indolent primary small intestinal CD4+ T-cell lymphomas. All patients presented with diarrhea and weight loss and were diagnosed with celiac disease refractory to a gluten free diet at referring institutions. Small intestinal biopsies showed crypt hyperplasia, villous atrophy and a dense lamina propria infiltrate of small-sized CD4+ T-cells often with CD7 downregulation or loss. Gastric and colonic involvement was also detected (n?=?2 each). Persistent, clonal TCR? gene rearrangement products were detected at multiple sites. SNP array analysis showed relative genomic stability, early in disease course, and non-recurrent genetic abnormalities, but complex changes were seen at disease transformation (n?=?1). Two patients are alive with persistent disease (4.6 and 2.5 years post-diagnosis), despite immunomodulatory therapy; one died due to bowel perforation related to large cell transformation 11 years post-diagnosis. Unique pathobiologic features warrant designation of indolent small intestinal CD4+ T-cell lymphoma as a distinct entity, greater awareness of which would avoid misdiagnosis as EATL or an inflammatory disorder, especially celiac disease.

SUBMITTER: Margolskee E

PROVIDER: S-EPMC3701677 | biostudies-literature | 2013

REPOSITORIES: biostudies-literature

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Indolent small intestinal CD4+ T-cell lymphoma is a distinct entity with unique biologic and clinical features.

Margolskee Elizabeth E Jobanputra Vaidehi V Lewis Suzanne K SK Alobeid Bachir B Green Peter H R PH Bhagat Govind G

PloS one 20130704 7

Enteropathy-associated T-cell lymphomas (EATL) are rare and generally aggressive types of peripheral T-cell lymphomas. Rare cases of primary, small intestinal CD4+ T-cell lymphomas with indolent behavior have been described, but are not well characterized. We describe morphologic, phenotypic, genomic and clinical features of 3 cases of indolent primary small intestinal CD4+ T-cell lymphomas. All patients presented with diarrhea and weight loss and were diagnosed with celiac disease refractory to ...[more]

PMID: 23861889

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Dataset Information

Indolent small intestinal CD4+ T-cell lymphoma is a distinct entity with unique biologic and clinical features.

Publications

Indolent small intestinal CD4+ T-cell lymphoma is a distinct entity with unique biologic and clinical features.

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