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T-cell Receptor Gene Rearrangement in T-cell Large Granular Leukocyte Leukemia : Preferential Var but Diverse Jar Usage in One of Five Patients

T-7 lymphoproliferative disease (T-7 LPD) is a chronic disorder of mature T cells that is associated with neutropenia and autoimmune phenomena. Although the progression of the lymphoproliferation is indolent, it is often associated with a monoclonal proliferation of T-cell-type large granular lymphocytes (LGL) that manifest multiple in vitro suppressor and cytotoxic activities. We considered the possibility that the granulocytopenia or anemia might represent an autoimmune disorder mediated by the monoclonal LGL via T-cell receptor (TCR) recognition of an antigen involved in hematopoiesis. Therefore, in an effort to characterize the usage of the TCR aand @-chain genes in patients with T-7 LPD, we cloned and sequenced TCR aand @-chain mRNAs derived from the T-cell type LGL of five patients. The five patients studied did not use a common Va nor a common J a segment. However, an unusual finding was observed in one of the patients where the occurrence of a single vari-