[Pure white cell aplasia (PWCA) with an inhibitor against colony-forming unit of granulocyte-macrophage (CFU-GM)].
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.
Iida
S
S
Department of Haematology, Shizuoka Saiseikai Sogo Hospital.
Noda
T
T
Banno
S
S
Nitta
M
M
Takada
K
K
Yamamoto
M
M
D000328:Adult; D000380:Agranulocytosis / Q000097:blood* / Q000276:immunology / Q000628:therapy; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000037:antagonists & inhibitors*; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007958:Leukocyte Count; D008297:Male; D010956:Plasmapheresis; D011994:Recombinant Proteins / Q000627:therapeutic use
D007070:Immunoglobulin A; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D016178:Granulocyte-Macrophage Colony-Stimulating Factor