Betticher D C, Pugin P
Service d'hématologie, Hôpital cantonal, Fribourg.
Schweiz Med Wochenschr. 1993 Jan 23;123(3):67-9.
A patient with idiopathic autoimmune hemolytic anemia is described. On day 5 of therapy with corticosteroids, immunoglobulin and several plasmaphereses, a high level of methemalbumin was found while RBC lysis had ceased and bilirubin and haptoglobin had normalized. The hemoglobin and the reticulocyte count were still very low and a bone marrow aspirate showed erythroaplasia. Since the LDH was consistently elevated during the entire period of erythroaplasia, ongoing intramedullary hemolysis due to antibodies (IgG) was assumed. The patient received cyclophosphamide. After 15 days all symptoms, signs of hemolysis and aplasia resolved. The patient has not had any other hemolytic crisis without treatment (follow-up 18 months). The presence of methemalbumin is observed in intravascular hemolysis and also in association with hemorrhagic pancreatitis. Its diagnostic and prognostic value is discussed.
描述了一名患有特发性自身免疫性溶血性贫血的患者。在使用皮质类固醇、免疫球蛋白并进行多次血浆置换治疗的第5天，发现高铁血红蛋白水平升高，而红细胞溶解已停止，胆红素和触珠蛋白已恢复正常。血红蛋白和网织红细胞计数仍然很低，骨髓穿刺显示红细胞生成异常。由于在红细胞生成异常的整个期间乳酸脱氢酶持续升高，推测存在由抗体（IgG）引起的持续性骨髓内溶血。该患者接受了环磷酰胺治疗。15天后，所有症状、溶血和发育不全的体征均消失。该患者未经治疗未发生任何其他溶血危机（随访18个月）。高铁血红蛋白在血管内溶血以及与出血性胰腺炎相关的情况下均可观察到。讨论了其诊断和预后价值。
