脂肪栓塞综合征继发于类固醇治疗镰状细胞血管闭塞危象1例。

IF 0.7 Q4 HEMATOLOGY Case Reports in Hematology Pub Date : 2023-01-01 DOI:10.1155/2023/5530870
Ram Prakash Thirugnanasambandam, Farish Mohamed Maraikayar, Marie Liu, Khalid Elbashir, John Muthu
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摘要

脂肪栓塞综合征(FES)通常被认为是骨折的并发症,已知会导致呼吸衰竭、皮肤皮疹、血小板减少症和神经损伤。非创伤性FES是罕见的,发生于骨髓坏死。在镰状细胞患者继发于类固醇治疗的血管闭塞危机是一个罕见的实体和不被广泛承认。我们报告一例FES继发于顽固性偏头痛患者的类固醇治疗。FES是一种罕见但严重的并发症,由于骨髓坏死而发生,通常与存活患者死亡率增加或破坏性神经系统后遗症有关。我们的病人最初因难治性偏头痛入院,并排除了任何急性紧急情况。随后,她的偏头痛在最初的治疗中没有得到缓解,医生给了她类固醇。她的病情恶化,并出现呼吸衰竭和精神状态改变,需要在重症监护室(ICU)进行护理。影像学检查显示微出血遍及大脑半球、脑干和小脑。肺部影像学证实她患有严重的急性胸综合征。患者也有肝细胞和肾脏损伤,表明多器官功能衰竭。患者接受了红细胞交换输血(RBCx)治疗,几天后几乎完全康复。然而,患者有残余的神经系统后遗症,存在麻木下巴综合征(NCS)。因此,本报告强调有必要认识到类固醇治疗继发的潜在多器官衰竭,以及开始红细胞交换输注治疗以降低类固醇继发并发症风险的重要性。
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Fat Embolization Syndrome Secondary to Steroid Treatment in a Case of Sickle Cell Vaso-Occlusive Crisis.

Fat embolization syndrome (FES) is often seen as a complication of fractures and has been known to cause respiratory failure, rashes of the skin, thrombocytopenia, and neurological damage. Nontraumatic FES is uncommon and occurs due to bone marrow necrosis. Vaso-occlusive crisis in sickle cell patients secondary to steroid therapy is a rare entity and not widely acknowledged. We report a case of FES secondary to steroid therapy administered for a patient with intractable migraine. FES is an uncommon yet serious complication that occurs due to bone marrow necrosis and is usually associated with increased mortality or damaging neurologic sequelae for the surviving patient. Our patient was initially admitted for intractable migraine and worked up to rule out any acute emergency conditions. She was then given steroids for her migraine which did not subside with the initial treatment. Her condition worsened, and she developed respiratory failure along with altered mental status requiring care in the intensive care unit (ICU). Imaging studies showed microhemorrhages throughout the cerebral hemispheres, brainstem, and cerebellum. The imaging of her lungs confirmed severe acute chest syndrome. The patient also had hepatocellular and renal injuries indicative of multiorgan failure. The patient was treated with a red cell exchange transfusion (RBCx) leading to an almost complete recovery in a few days. The patient, however, had residual neurological sequelae with the presence of numb chin syndrome (NCS). This report thus highlights the need to recognize potential multiorgan failure secondary to steroid treatment and the importance of initiating treatment with red cell exchange transfusions to decrease the risk of such complications secondary to steroids.

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