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 (Tγ 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, CDS, HNK‐1 monoclonal antibodies and of cytochemical stains (acid phosphatase and alpha‐naphthyl butyrate esterase) for characterization of this disorder is discussed.
- large granular T‐cell lymphoproliferative disorder
- natural killer cell leukemia
- suppressor cell leukemia
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