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(American Journal of Pathology. 1998;153:1483-1490.)
© 1998 American Society for Investigative Pathology

Regular Articles

Most CD56⁺ Intestinal Lymphomas Are CD8⁺CD5^- T-Cell Lymphomas of Monomorphic Small to Medium Size Histology

Andreas Chott* , Wolfgang Haedicke , Isabella Mosberger* , Manuela Födinger , Karin Winkler , Christine Mannhalter and Hans-Konrad Müller-Hermelink

From the Departments of Clinical Pathology* and Laboratory Medicine, University of Vienna, Vienna, Austria, and the Department of Pathology, University of Würzburg, Würzburg, Germany

The expression of the natural killer (NK) cell marker CD56 has been reported to occur in NK cell lymphomas/leukemias and a small group of peripheral T-cell lymphomas but has not been studied extensively in primary intestinal non-B-cell lymphomas. Normal human jejunal intraepithelial lymphocytes (IELs) are mainly T-cell receptor (TCR)-ß⁺CD3⁺CD8⁺CD5^low and include an ~15% fraction of CD56⁺ cells that could be the cells of origin for CD56⁺ intestinal T-cell lymphoma (ITL). To test this hypothesis, 70 cases diagnosed as ITL were immunophenotyped, and 15 CD56⁺ cases (21%) were identified. The majority of the CD56⁺ lymphomas was of monomorphic small to medium-sized histology, shared the common phenotype ßF1^±CD3/cyt⁺CD8⁺CD4^-CD5^-CD57^-TIA-1⁺ and had clonally rearranged TCR -chain genes. In contrast, the CD56^- lymphomas were mainly composed of pleomorphic medium and large cells or had a morphology most consistent with anaplastic large-cell lymphoma and were mostly CD8^-. These findings suggest that the majority of CD56⁺ intestinal lymphomas are morphologically and phenotypically distinct T-cell lymphomas most likely derived from activated cytotoxic CD56⁺CD8⁺ IELs. Some overlapping histological and clinical features between CD56⁺ and CD56^- ITLs indicate that the former belong to the clinicopathological entity of ITL. The consistent expression of cytotoxic-granule-associated proteins introduces ITL (both CD56⁺ and CD56^-) into the growing family of usually aggressive extranodal lymphomas of cytotoxic T-cell and NK-cell derivation. In contrast to putative NK-cell lymphoma of the sinonasal region, intestinal NK-cell lymphoma seems to be very rare.

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