CD3+, CD4−, CD8− large granular T-cell lymphoproliferative disorder
- 1 July 1991
- journal article
- case report
- Published by Wiley in American Journal of Hematology
- Vol. 37 (3), 173-178
- https://doi.org/10.1002/ajh.2830370308
Abstract
Large granular T-cell lymphoproliferative disorder (LGTLD) is a heterogeneous disorder covering a broad spectrum of diseases and requiring further subdivision. Most reported cases emphasized its suppressor phenotype (T gamma cell or CD8+), but we encountered two cases of CD3+, CD4-, CD8- LGTLD. Both cases had a benign clinical course and required no chemotherapy despite persistent lymphocytosis. This unique phenotype has been reported in a few cases of acute lymphoblastic leukemia expressing the T-cell receptor (TcR) gamma chain gene and is considered the counterpart of thymocytes at the intermediate stage between early precursors and mature thymocytes. Our case 1 provides further evidence that the CD3+, CD4-, CD8- phenotype, indeed, expresses the TcR gamma chain gene. However, the negative reaction to terminal deoxynucleotidyl transferase in our case 1 indicates that this phenotype represents proliferation of peripheral T-cells, in which about 2% bear the CD3+, CD4-, CD8- phenotype in the normal population. The selective use of CD3, CD4, CD8, HNK-1 monoclonal antibodies and of cytochemical stains (acid phosphatase and alpha-naphthyl butyrate esterase) for characterization of this disorder is discussed.Keywords
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