Roush G R, Rosenthal N S, Gerson S L, Toy E M, McCarthy P, Hirschler N V, Yomtovian R
Department of Pathology, Case Western Reserve University, Cleveland, Ohio, USA.
Transfusion. 1996 Jun;36(6):575-80. doi: 10.1046/j.1537-2995.1996.36696269519.x.
Autoantibodies with anti-U specificity, usually in combination with autoantibodies of other specificities, have occasionally been identified in association with autoimmune hemolytic anemia. A case of life-threatening autoimmune hemolytic anemia, characterized by several atypical features, including apparent intravascular hemolysis associated with an IgG2 anti-U, reticulocytopenia, and bone marrow dyserythropoiesis is described.
A 36-year-old man with a severe case of acute-onset autoimmune hemolytic anemia was admitted to another hospital; he had a hematocrit of 15 percent, elevated bilirubin and lactate dehydrogenase, and positive direct and indirect antiglobulin tests. He received 7 units of incompatible red cells without improvement in hematocrit, and he was transferred to University Hospitals of Cleveland (OH). He was jaundiced and became syncopal in the sitting position. His serum was reddish pink; he had a hematocrit of 11.8 percent and a reticulocyte count of 2.5 percent. No spherocytes were observed in the peripheral blood smear. Shortly after admission, the hematocrit fell to 6.9 percent. He was given 3 units of "least-incompatible" red cells and was started on prednisone, with little improvement. An IgG2 autoanti-U was detected in his serum. Seven units of U- red cells were transfused over the next 4 days. The hematocrit improved to 23 percent and continued to rise without further transfusion. A bone marrow examination, initially revealing erythroid hyperplasia accompanied by dyserythropoiesis, became morphologically normal. Drug studies failed to show evidence of drug-related hemolysis. He remains well 2 years after discharge without evidence of recurrent hemolysis.
Severe life-threatening autoimmune hemolytic anemia, in this instance induced by an autoanti-U, may be associated with IgG2 autoantibody and characterized by apparent intravascular hemolysis and bone marrow dyserythropoiesis. Early treatment with U- blood, in addition to steroids, may be beneficial.
具有抗-U特异性的自身抗体，通常与其他特异性的自身抗体联合出现，偶尔在自身免疫性溶血性贫血中被发现。本文描述了一例危及生命的自身免疫性溶血性贫血病例，其具有几个非典型特征，包括与IgG2抗-U相关的明显血管内溶血、网织红细胞减少和骨髓红系造血异常。
一名36岁男性因严重急性发作的自身免疫性溶血性贫血入住另一家医院；其血细胞比容为15%，胆红素和乳酸脱氢酶升高，直接和间接抗球蛋白试验均为阳性。他接受了7单位不相容红细胞输注，但血细胞比容无改善，随后被转至克利夫兰大学医院（俄亥俄州）。他有黄疸，坐位时出现晕厥。其血清呈淡红色；血细胞比容为11.8%，网织红细胞计数为2.5%。外周血涂片未观察到球形红细胞。入院后不久，血细胞比容降至6.9%。他接受了3单位“最不相容性最小”的红细胞输注，并开始使用泼尼松治疗，但改善甚微。在他的血清中检测到IgG2自身抗-U。在接下来的4天内输注了7单位U-红细胞。血细胞比容升至23%，且在未进一步输血的情况下持续上升。骨髓检查最初显示红系增生伴红系造血异常，之后形态学恢复正常。药物研究未发现药物相关溶血的证据。出院2年后他情况良好，无溶血复发迹象。
严重危及生命的自身免疫性溶血性贫血，在本例中由自身抗-U引起，可能与IgG2自身抗体相关，其特征为明显的血管内溶血和骨髓红系造血异常。除了使用类固醇外，早期输注U-血可能有益。
