Menakuru Sasmith R, Priscu Adelina, Dhillon Vijaypal, Salih Ahmed
Internal Medicine, Indiana University Health, Ball Memorial Hospital, Muncie, IN 47303, USA.
Hematol Rep. 2022 Jul 21;14(3):235-239. doi: 10.3390/hematolrep14030032.
Patients with sickle cell anemia often receive multiple red blood cell (RBC) transfusions during their lifetime. Hyperhemolysis is a life-threatening phenomenon of accelerated hemolysis and worsening anemia that occurs when both transfused RBCs and autologous RBCs are destroyed. The level of hemoglobin post-transfusion is lower than pre-transfusion levels, and patients are usually hemodynamically unstable. Hyperhemolysis must be differentiated from a delayed hemolytic transfusion reaction during which destruction of transfused RBC is the cause of anemia. Hyperhemolysis syndrome can be differentiated into acute (within seven days) and chronic forms (after seven days) post-transfusion. The authors present a case of acute hyperhemolysis syndrome in a patient with sickle cell anemia refractory to steroids and IVIG, which are the treatment of choice. The patient was treated with tocilizumab, combined with supportive measures of erythropoietin, iron, vitamin B12, and folate.
镰状细胞贫血患者一生中常接受多次红细胞（RBC）输血。高溶血是一种危及生命的现象，表现为加速溶血和贫血加重，当输入的红细胞和自身红细胞均被破坏时就会发生。输血后血红蛋白水平低于输血前水平，且患者通常血流动力学不稳定。高溶血必须与迟发性溶血性输血反应相鉴别，后者中输入红细胞的破坏是贫血的原因。高溶血综合征可分为输血后急性（7天内）和慢性（7天后）两种形式。作者报告了1例镰状细胞贫血患者发生急性高溶血综合征的病例，该患者对类固醇和静脉注射免疫球蛋白（IVIG）（治疗的首选药物）难治。该患者接受了托珠单抗治疗，并结合了促红细胞生成素、铁、维生素B12和叶酸的支持性措施。
