Tsitsikas Dimitris A, Vize Jessica, Abukar Jibril
Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK.
Research and Innovation Department, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK.
J Clin Med. 2020 Nov 8;9(11):3601. doi: 10.3390/jcm9113601.
Fat embolism syndrome is a devastating complication of sickle cell disease resulting from extensive bone marrow necrosis and associated with high mortality rates, while survivors often suffer severe neurological sequelae. Despite that, the syndrome remains under-recognised and under-diagnosed. Paradoxically, it affects exclusively patients with mild forms of sickle cell disease, predominantly HbSC and HbSβ. A significant number of cases occur in the context of human parvovirus B19 infection. We provide here a brief summary of the existing literature and describe our experience treating 8 patients in our institution. One patient had HbSS, 6 HbSC and 1 HbSβ. All patients developed type I respiratory failure and neurological involvement either at presentation or within the first 72 h. The most striking laboratory abnormality was a 100-fold increase of the serum ferritin from baseline. Seven patients received emergency red cell exchange and 1 simple transfusion. Two patients (25%) died, 2 patients (25%) suffered severe neurological impairment and 1 (12%) mild neurological impairment on discharge, while 3 (38%) patients made a complete recovery. With long-term follow-up, 1 patient with severe neurological impairment and one patient with mild neurological impairment made dramatic improvements, making the long-term complete recovery or near complete recovery rate 63%. Immediate red cell exchange transfusion can be lifesaving and should be instituted as soon as the syndrome is suspected. However, as the outcomes remain unsatisfactory despite the increasing use of red cell exchange, we suggest additional therapeutic measures such as therapeutic plasma exchange and pre-emptive transfusion for high risk patients.
脂肪栓塞综合征是镰状细胞病的一种严重并发症，由广泛的骨髓坏死引起，死亡率高，幸存者常伴有严重的神经后遗症。尽管如此，该综合征仍未得到充分认识和诊断。矛盾的是，它仅影响轻度镰状细胞病患者，主要是HbSC和HbSβ型。大量病例发生在人类细小病毒B19感染的背景下。我们在此简要总结现有文献，并描述我们在本机构治疗8例患者的经验。1例患者为HbSS型，6例为HbSC型，1例为HbSβ型。所有患者在就诊时或最初72小时内均出现I型呼吸衰竭和神经受累。最显著的实验室异常是血清铁蛋白较基线水平升高了100倍。7例患者接受了紧急红细胞置换，1例接受了单纯输血。2例患者（25%）死亡，2例患者（25%）出院时出现严重神经功能障碍，1例（12%）出现轻度神经功能障碍，3例（38%）患者完全康复。经过长期随访，1例严重神经功能障碍患者和1例轻度神经功能障碍患者有显著改善，长期完全康复或接近完全康复率为63%。立即进行红细胞置换输血可能挽救生命，一旦怀疑该综合征应立即实施。然而，尽管红细胞置换的使用越来越多，但结果仍不尽人意，我们建议采取额外的治疗措施（如治疗性血浆置换）以及对高危患者进行预防性输血。
