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 c...

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Veröffentlicht in:Blood 1986-05, Vol.67 (5), p.1427-1432
Hauptverfasser: Winton, Elliott F., Chan, Wing C., Check, Irene, Colenda, Kathryn W., Bongiovanni, Kathleen F., Waldmann, Thomas A.
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Sprache:eng
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Zusammenfassung: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.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood.V67.5.1427.1427