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一名镰状细胞病血管闭塞危象后发生骨髓坏死的患者,因推测存在广泛脂肪栓子导致脑微出血。

Brain microbleeds resulting from presumed extensive fat emboli in a patient with bone marrow necrosis following a sickle cell disease vaso-occlusive crisis.

作者信息

Amseian Gary, Ortiz-Fernández Maria, Doti Pamela, Massuet Anna, Castro Pedro, Pineda Camilo

机构信息

Radiology Department, Hospital Clínic de Barcelona, C. de Villarroel, 170, Barcelona, 08036, Spain.

Internal Medicine Department, Hospital Clínic de Barcelona, Barcelona, 08036, Spain.

出版信息

Emerg Radiol. 2025 Feb;32(1):125-129. doi: 10.1007/s10140-024-02283-5. Epub 2024 Sep 28.

Abstract

Acute manifestations of sickle cell disease (SCD) are numerous and multisystemic. Cerebral fat embolism (CFE) is a rare but serious complication of SCD caused by bone marrow necrosis (BMN) during vaso-occlusive crises (VOC). We present the case of a 41-year-old man with SCD who developed severe VOC and multi-organ dysfunction. He subsequently experienced neurological deterioration with decreased consciousness and diffuse encephalopathy on serial electroencephalograms. Bone marrow aspiration confirmed BMN. Brain MRI revealed extensive diffuse leukoencephalopathy, vasogenic and cytotoxic edema in the white matter, patchy edema in the cranial vault bone marrow on fat-suppressed FLAIR sequence (a finding consistent with the confirmed BMN), and multiple cerebral microbleeds on susceptibility-weighted imaging consistent with CFE. The management of acute neurological complications of SCD varies depending on the specific complication. Brain MRI plays a crucial role in the accurate diagnosis of these complications to guide appropriate treatment.

摘要

镰状细胞病(SCD)的急性表现多种多样且累及多系统。脑脂肪栓塞(CFE)是SCD的一种罕见但严重的并发症,由血管闭塞性危机(VOC)期间的骨髓坏死(BMN)引起。我们报告一例41岁患有SCD的男性患者,该患者发生了严重的VOC和多器官功能障碍。随后,他出现神经功能恶化,意识下降,连续脑电图显示弥漫性脑病。骨髓穿刺证实存在BMN。脑部MRI显示广泛的弥漫性白质脑病、白质中的血管源性和细胞毒性水肿、脂肪抑制FLAIR序列上颅顶骨髓的斑片状水肿(这一发现与确诊的BMN一致),以及磁敏感加权成像上与CFE一致的多个脑微出血灶。SCD急性神经并发症的治疗因具体并发症而异。脑部MRI在准确诊断这些并发症以指导适当治疗方面起着关键作用。

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