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L Degos, A Faille, M Housset, L Boumsell, C Rabian and T Parames
The clinical, hematologic, and immunologic findings of a syndrome of
agranulocytosis, hypogammaglobulinemia, and thymoma are described.
Neutrophil agranulocytosis predisposing to severe infectious disease
resulted from a deficiency of mature cells in bone marrow. Autologous and
heterologous stem cell growth in vitro was inhibited by the patient's
serum. Immunoglobulin deficiency was secondary to the absence of peripheral
blood B lymphocytes, while T-cell subpopulations and cellular immunity were
present. Surgical removal of a spindle cell thymoma had no effect on the
agranulocytosis and B-cell deficiency. The hematologic findings did not
respond to steroid therapy and cyclophosphamide. However, the
agranulocytosis improved with repeated plasmapheresis and the patient
achieved a clinical remission.
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| Copyright © 1982 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||