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  • Title: [Pure white cell aplasia (PWCA) with an inhibitor against colony-forming unit of granulocyte-macrophage (CFU-GM)].
    Author: Iida S, Noda T, Banno S, Nitta M, Takada K, Yamamoto M.
    Journal: Rinsho Ketsueki; 1990 Oct; 31(10):1726-30. PubMed ID: 1701503.
    Abstract:
    We reported here a case of pure white cell aplasia (PWCA). A 23-year-old man was admitted to our hospital in September 1989 because of agranulocytosis, fever, and anal pain. He had no history of toxic-drug exposure or blood transfusion. Laboratory studies were all within the normal range except white blood cell count of 2,300/microliters with no neutrophils and low serum IgA level (28 mg/dl). Bone marrow examination showed hypocellular with erythroid predominance and no granulocyte maturation beyond the myelocyte. Complement-dependent suppression of autologous and heterologous granulocyte-macrophage colony-forming units by the patient's serum could be demonstrated. Though corticosteroid administration was ineffective, neutropenia improved by plasmapheresis. Furthermore, recombinant granulocyte colony stimulating factor (rG-CSF) could release him from persistent bacterial infection of anal fistula by transient improvement of neutropenia. These findings suggest a humoral autoimmune mechanism for the pathogenesis of PWCA and the effectiveness of rG-CSF for the patient with severe infections.
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