Kalter Joshua A, Gupta Ranju, Greenberg Marna Rayl, Miller Andrew J, Allen Jamie
USF Morsani College of Medicine, Lehigh Valley Health Network, Department of Emergency and Hospital Medicine, Allentown, Pennsylvania.
USF Morsani College of Medicine, Lehigh Valley Health Network, Division of Hematology Oncology, Allentown, Pennsylvania.
Clin Pract Cases Emerg Med. 2021 Feb;5(1):101-104. doi: 10.5811/cpcem.2020.12.50349.
Hyperhemolysis syndrome (HHS) is a rare complication of repeat blood transfusions in sickle cell disease (SCD). This can occur acutely or have a delayed presentation and often goes unrecognized in the emergency department (ED) due to its rapid progression and similarity to acute chest syndrome and other common complications of SCD.
We present a case of a 20-year-old male with SCD who presented to the ED with pain and tenderness in his lower extremities one day after discharge for a crisis. Unbeknownst to the ED team, during his admission he had received a blood transfusion. On presentation he was noted to have hyperkalemia, hyperbilirubinemia, anemia, and uncontrolled pain, and was admitted for sickle cell pain crisis. Over the next 36 hours, his hemoglobin dropped precipitously from 8.9 grams per deciliter (g/dL) to 4.2 g/dL (reference range: 11.5-14.5 g/dL), reticulocyte count from 11.7 % to 3.8% (0.4-2.2%), and platelets from 318,000 per cubic centimeter (K/cm) to 65 K/cm (140-350 K/cm). He also developed a fever, hypoxia, transaminitis, a deteriorating mental status, and severe lactic acidosis. Hematology was consulted and he was treated with methylprednisolone, intravenous immunoglobulin, two units of antigen-matched red blood cells, fresh frozen plasma, and cryoprecipitate. He was transferred to an outside hospital for exchange transfusion and remained hospitalized for 26 days with acute liver failure, bone marrow necrosis, and a fever of unknown origin.
Because of the untoward outcomes associated with delay in HHS diagnosis and the need for early initiation of steroids, it is important for emergency providers to screen patients with hemoglobinopathies for recent transfusion at ED presentation. Asking the simple question about when a patient's last transfusion occurred can lead an emergency physician to include HHS in the differential and work-up of patients with sickle cell disease complications.
高溶血综合征（HHS）是镰状细胞病（SCD）反复输血后的一种罕见并发症。其可急性发生或延迟出现，由于进展迅速且与急性胸部综合征及SCD的其他常见并发症相似，在急诊科（ED）常未被识别。
我们报告一例20岁的SCD男性患者，在因一次危象出院一天后因下肢疼痛和压痛就诊于急诊科。急诊科团队并不知晓，其住院期间接受了输血。就诊时发现他有高钾血症、高胆红素血症、贫血且疼痛未得到控制，因镰状细胞疼痛危象入院。在接下来的36小时内，他的血红蛋白从每分升8.9克（g/dL）急剧降至4.2 g/dL（参考范围：11.5 - 14.5 g/dL），网织红细胞计数从11.7%降至3.8%（0.4 - 2.2%），血小板从每立方厘米318,000（K/cm）降至65 K/cm（140 - 350 K/cm）。他还出现发热、低氧血症、转氨酶升高、精神状态恶化及严重乳酸酸中毒。血液科会诊后，给予他甲泼尼龙、静脉注射免疫球蛋白、两单位抗原匹配的红细胞、新鲜冰冻血浆和冷沉淀治疗。他被转至外院进行换血治疗，因急性肝衰竭、骨髓坏死及不明原因发热住院26天。
由于HHS诊断延迟会带来不良后果且需要早期使用类固醇，急诊科医护人员在患者就诊时对血红蛋白病患者筛查近期输血情况很重要。询问患者最后一次输血时间这个简单问题，可使急诊医生在鉴别诊断和检查镰状细胞病并发症患者时考虑到HHS。
