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[暴发性自身免疫性冷型溶血伴单热性冷凝集素显著升高。膜式血浆置换治疗成功]

[Fulminant autoimmune cold-type hemolysis with marked elevation of monothermic cold agglutinins. Successful therapy with membrane plasmapheresis].

作者信息

Gordjani N, Kretschmer V, Töllner U, Rieger C

机构信息

Universitäts-Kinderklinik Marburg.

出版信息

Monatsschr Kinderheilkd. 1990 Aug;138(8):454-6.

PMID:2215510
Abstract

Autoimmune hemolytic anemia associated with cold autoantibodies is rare in infancy. In a 7 months old infant with severe hemolysis, a hemoglobin of 5.9 g/dl and a cold agglutinin titer of 1:8000, even the transfusion of warmed, packed red cells (37 degrees C) lead to hemolysis. Hemoglobin fell to 2.8 g/dl despite prevention of exposure to cold, parenteral steroids and immunoglobulins. Cold agglutinin titer fell to 1:8 after plasma separation. Subsequent transfusion did not lead to hemolysis and permanent remission was achieved.

摘要

与冷凝集素相关的自身免疫性溶血性贫血在婴儿期较为罕见。一名7个月大的严重溶血婴儿,血红蛋白为5.9 g/dl,冷凝集素效价为1:8000,即使输注预热至37摄氏度的浓缩红细胞也会导致溶血。尽管避免接触寒冷、使用肠外类固醇和免疫球蛋白,血红蛋白仍降至2.8 g/dl。血浆置换后冷凝集素效价降至1:8。随后的输血未导致溶血,患儿实现了永久缓解。

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