Thirugnanasambandam Ram Prakash, Maraikayar Farish Mohamed, Liu Marie, Elbashir Khalid, Muthu John
Department of Internal Medicine, SUNY Downstate Medical Center, Brooklyn, NY 11203, USA.
Medical Student, SUNY Downstate Medical Center, Brooklyn, NY 11203, USA.
Case Rep Hematol. 2023 Jul 3;2023:5530870. doi: 10.1155/2023/5530870. eCollection 2023.
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.
脂肪栓塞综合征(FES)常被视为骨折的一种并发症,已知可导致呼吸衰竭、皮肤皮疹、血小板减少和神经损伤。非创伤性FES并不常见,是由骨髓坏死引起的。镰状细胞病患者在接受类固醇治疗后继发血管闭塞性危象是一种罕见情况,尚未得到广泛认可。我们报告一例因对一名顽固性偏头痛患者进行类固醇治疗继发FES的病例。FES是一种罕见但严重的并发症,由骨髓坏死引起,通常与死亡率增加或存活患者出现神经损伤后遗症有关。我们的患者最初因顽固性偏头痛入院,接受检查以排除任何急性紧急情况。随后因偏头痛给予她类固醇治疗,但初始治疗后偏头痛并未缓解。她的病情恶化,出现呼吸衰竭以及精神状态改变,需要在重症监护病房(ICU)接受治疗。影像学检查显示整个大脑半球、脑干和小脑有微出血。她肺部的影像学检查证实有严重急性胸部综合征。患者还存在肝细胞和肾损伤,提示多器官功能衰竭。该患者接受了红细胞置换输血(RBCx)治疗,几天后几乎完全康复。然而,患者仍有残留神经后遗症,存在麻木性颏部综合征(NCS)。因此,本报告强调了认识类固醇治疗继发潜在多器官功能衰竭的必要性,以及启动红细胞置换输血治疗以降低类固醇继发此类并发症风险的重要性。