Dabrow M B, Wilkins J C
Postgrad Med. 1993 Apr;93(5):183-90. doi: 10.1080/00325481.1993.11701668.
Two hematologic emergencies are reviewed in this article: transfusion reactions and crises in patients who have sickle cell disease. Transfusion reactions may be due to incompatibility, IgA deficiency, allergy or, rarely, bacterial contamination of the blood product. A major hemolytic reaction due to incompatibility may progress to hypotension and shock. To prevent this type of reaction, blood products should be given only when necessary and attention should be given to eliminating clerical errors, which are responsible for many hemolytic reactions. In patients with sickle cell disease, a painful crisis due to vascular occlusion is the most common emergency. Rehydration is essential, and narcotics may be needed to relieve pain. Aplastic crisis is managed by transfusion of packed red blood cells and supportive care. Sickle cell crisis may affect major organ systems. The acute chest syndrome can be complicated by pneumonia; rapid respiratory failure may occur if multiple lobes are involved. Splenic or hepatic sequestration requires aggressive rehydration and transfusion. In patients who have had stroke or subarachnoid hemorrhage, a long-term exchange transfusion program is needed to keep hemoglobin S levels below 30%.
输血反应和镰状细胞病患者的危象。输血反应可能是由于血型不合、IgA缺乏、过敏，或极少情况下血制品的细菌污染所致。由血型不合引起的严重溶血反应可能进展为低血压和休克。为预防此类反应，仅在必要时输注血制品，并应注意消除文书错误，文书错误是许多溶血反应的原因。在镰状细胞病患者中，血管闭塞引起的疼痛危象是最常见的急症。补液至关重要，可能需要使用麻醉剂来缓解疼痛。再生障碍危象通过输注浓缩红细胞和支持性治疗来处理。镰状细胞危象可能影响主要器官系统。急性胸综合征可能并发肺炎；如果多个肺叶受累，可能会迅速发生呼吸衰竭。脾或肝的血液滞留需要积极补液和输血。对于曾发生中风或蛛网膜下腔出血的患者，需要进行长期的换血治疗方案，以使血红蛋白S水平低于30%。
