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

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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
StatePublished - May 1 1992

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Peripheral T-Cell Lymphoma
Neural Cell Adhesion Molecules
T-cells
Nasopharynx
Polymerase chain reaction
RNA-Directed DNA Polymerase
Neurology
Reverse Transcriptase Polymerase Chain Reaction
Muscle
Gastrointestinal Tract
Pancreas
Assays
Lymphoma
Thyroid Gland
Central Nervous System
Genes
RNA
Tissue
Muscles
Antibodies

ASJC Scopus subject areas

  • Hematology

Cite this

Neural cell adhesion molecule-positive peripheral T-cell lymphoma : A rare variant with a propensity for unusual sites of involvement. / Kern, W. F.; Spier, C. M.; Hanneman, E. H.; Miller, T. P.; Matzner, M.; Grogan, T. M.

In: Blood, Vol. 79, No. 9, 01.05.1992, p. 2432-2437.

Research output: Contribution to journalArticle

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N2 - 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."

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