Spontaneous regression of a monoclonal proliferation of large granular lymphocytes associated with reversal of anemia and neutropenia.
A 43-year-old male with a phenotypically homogeneous, expanded subset of T cells presented in 1981 with anemia and neutropenia. The surface antigen phenotype of 99% of the peripheral blood lymphocytes was T3+, T8+, T4-, and they were morphologically large granular lymphocytes (LGL). The same cells comprised 37% of the marrow nucleated cells. Eight months after he presented, the peripheral blood T8+, LGL diminished spontaneously, and the anemia and neutropenia completely resolved. The patient remains hematologically normal as of October 1984. To determine if the T8+, LGL represented a clonal expansion, DNA from peripheral blood lymphocytes collected and cryopreserved when the patient was neutropenic and anemic, and when he was hematologically normal, was analyzed for clonal T-cell antigen receptor gene rearrangements. Using Southern blot analysis, a clonal DNA rearrangement was demonstrated, and this clone diminished but was still demonstrable in peripheral blood lymphocytes collected in 1984. The above observations implicate the expanded T8+, LGL in the pathogenesis of the neutropenia and anemia, yet the exact mechanism remains to be elucidated.
D000328:Adult; D000380:Agranulocytosis / Q000473:pathology*; D000740:Anemia / Q000150:complications / Q000235:genetics / Q000473:pathology*; D000954:Antigens, Surface / Q000032:analysis; D001772:Blood Cell Count; D002999:Clone Cells / Q000166:cytology*; D004247:DNA / Q000032:analysis; D006801:Humans; D008214:Lymphocytes / Q000166:cytology*; D008297:Male; D009503:Neutropenia / Q000150:complications / Q000235:genetics / Q000473:pathology*; D010641:Phenotype; D011948:Receptors, Antigen, T-Cell / Q000235:genetics; D013997:Time Factors
D000954:Antigens, Surface; D011948:Receptors, Antigen, T-Cell; D004247:DNA