Neural cell adhesion molecule-positive peripheral T-cell lymphoma: A rare variant with a propensity for unusual sites of involvement

W. F. Kern, C. M. Spier, E. H. Hanneman, T. P. Miller, M. Matzner, T. M. Grogan

Research output: Contribution to journalArticle

219 Scopus citations

Abstract

A distinct subset of patients with peripheral T-cell lymphoma (PTCL) is described which reacts with Leu-19 (CD56), an antibody that has been shown to identify the neural cell adhesion molecule (NCAM). These NCAM-positive PTCL patients (11 of a series of 46 PTCL; 24%) exhibited a striking predilection for unusual anatomic sites of involvement: central nervous system (36%), muscle (18%), gastrointestinal tract, and nasopharynx (27% each). Additional extranodal sites of involvement included the pituitary, thyroid, parathyroids, adrenals, and pancreas. The NCAM-positive subset also exhibited a characteristic phenotypic profile, with significantly lower expression of CD3 and CD5 compared with the NCAM-negative group. RNA transcripts consistent with the NCAM gene were detected in tissue samples from five Leu-19-positive cases using a reverse transcriptase-polymerase chain reaction assay, supporting the idea that Leu-19 recognizes NCAM in these patient samples. This suggests that the expression of the NCAM plays a role in the behavior and localization of lymphomas. Because of the unique clinical and phenotypic characteristics of this group it may be designated as 'NCAM-positive peripheral T-cell lymphoma.'

Original languageEnglish (US)
Pages (from-to)2432-2437
Number of pages6
JournalBlood
Volume79
Issue number9
DOIs
StatePublished - 1992

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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