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Neural cell adhesion molecule-positive peripheral T-cell lymphoma: a rare
variant with a propensity for unusual sites of involvement [see comments]
WF Kern, CM Spier, EH Hanneman, TP Miller, M Matzner and TM Grogan
Department of Pathology, University of Arizona, Tucson.
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."
Volume 79,
Issue 9,
pp. 2432-2437,
05/01/1992
Copyright © 1992 by The American Society of Hematology
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