Neural Cell Adhesion Molecule-Positive Peripheral T-Cell Lymphoma: A Rare Variant With a Propensity for Unusual Sites of Involvement

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 predilectio...

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Veröffentlicht in:Blood 1992-05, Vol.79 (9), p.2432-2437
Hauptverfasser: Kern, W.F., Spier, C.M., Hanneman, E.H., Miller, T.P., Matzner, M., Grogan, T.M.
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container_end_page 2437
container_issue 9
container_start_page 2432
container_title Blood
container_volume 79
creator Kern, W.F.
Spier, C.M.
Hanneman, E.H.
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Matzner, M.
Grogan, T.M.
description 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 signif-icantly 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-poly-merase 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.”
doi_str_mv 10.1182/blood.V79.9.2432.2432
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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 signif-icantly 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-poly-merase 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.”</abstract><cop>WASHINGTON</cop><pub>Elsevier Inc</pub><pmid>1373974</pmid><doi>10.1182/blood.V79.9.2432.2432</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record>
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source Web of Science - Science Citation Index Expanded - 1992<img src="https://exlibris-pub.s3.amazonaws.com/fromwos-v2.jpg" />; MEDLINE; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection
subjects Adult
Aged
Aged, 80 and over
Antigens, CD - analysis
Base Sequence
Biological and medical sciences
Cell Adhesion Molecules, Neuronal - analysis
Cell Adhesion Molecules, Neuronal - genetics
Female
Hematologic and hematopoietic diseases
Hematology
Humans
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Life Sciences & Biomedicine
Lymphoma, T-Cell, Peripheral - immunology
Lymphoma, T-Cell, Peripheral - metabolism
Lymphoma, T-Cell, Peripheral - pathology
Male
Medical sciences
Middle Aged
Molecular Sequence Data
Phenotype
RNA - analysis
Science & Technology
title Neural Cell Adhesion Molecule-Positive Peripheral T-Cell Lymphoma: A Rare Variant With a Propensity for Unusual Sites of Involvement
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