Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Refractory Anemia associated with Pure Red Cell Aplasia-like Syndrome and Various Autoantibodies after 7-Year Observation
Hisao FUJIMOTOKazuo OKADAHiroko OHIZUMISatoshi HASHINOMasanori TANAKAMasanobu KOBAYASHINagahito SAITOMasahiro IMAMURAKeisuke SAKURADATamotsu MIYAZAKI
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1988 Volume 29 Issue 5 Pages 760-764

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Abstract

We report here a case of refractory anemia (RA) associated with various antoantibodies and pure red cell aplasia (PRCA)-like syndrome. A 73 year-old man, who had suffered from RA since 1979, was admitted to our hospital in April, 1986, because of severe anemia. On admission, he had a remarkably elevated level of serum γ-globlin, a decreased level of serum haptoglobin, a high titer of antinuclear antibody, positive direct and indirect Coombs' tests, and a decreased number of erythroblasts in the bone marrow. A life span of red blood cells (51Cr T1/2) was shortened in May, 1986. Serum showed cytotoxicity against allogeneic lymphocytes. These abnormalities were not observed at the onset in 1979. Five months after the admission, he was administered with prednisolone (40mg/day) from September 1, 1986. Anemia and erythroid hypoplasia were gradually improved following this therapy. BFU-E and CFU-C were hardly detectable on admission. Although CFU-E of non-adherent cell fraction in November, 1986, was not yet observed, CFU-E of non-adherent and non-T cell fraction was 23/2×105 cells. Decreased OKT 4 positive lymphocytes and increased OKT8 positive lymphocytes in peripheral blood became to normal level after administration of steroid hormone. It was suggested that erythroid hypoplasia was closely related to the inhibition by suppressor cells against erythroid precursor cells, causing the disturbance of maturation and proliferation of these cells, and the involvement of hemolysis might be unlikely as the cause of anemia.

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© 1988 The Japanese Society of Clinical Hematology
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