Se Bobby, Frisch Austin, Hwang Min Woo, Polani Faran, Bade Najeebah
Inova Fairfax Hospital Department of Internal Medicine, Falls Church, VA 22042, USA.
Inova Schar Cancer Institute, Fairfax, VA 22031, USA.
J Hematol. 2024 Jun;13(3):104-107. doi: 10.14740/jh1274. Epub 2024 Jun 28.
Thrombotic microangiopathies cause ischemic organ damage and require urgent management for a favorable prognosis. Fat embolism syndrome from bone marrow necrosis is a rare and unique pathology that carries a high mortality rate. It can mimic thrombotic microangiopathies such as thrombotic thrombocytopenic purpura (TTP). Herein, we present a patient with sickle cell-beta-thalassemia who initially presented with a vaso-occlusive crisis, lab evidence of hemolysis, schistocytes and thrombocytopenia who developed acute encephalopathy with respiratory distress, consistent with TTP. She was found to have multiple infarcts in the brain. She was intubated and underwent plasma and red cell exchange. Bone marrow biopsy confirmed marrow necrosis from her vaso-occlusive crisis and subsequently, fat embolism syndrome. Here, we discuss the complex presentation and the complications of fat embolism from bone marrow necrosis and how it can mimic TTP.
血栓性微血管病可导致缺血性器官损伤，需要紧急处理以获得良好预后。骨髓坏死引起的脂肪栓塞综合征是一种罕见且独特的病理情况，死亡率很高。它可酷似血栓性微血管病，如血栓性血小板减少性紫癜（TTP）。在此，我们报告一名镰状细胞-β地中海贫血患者，最初表现为血管闭塞性危象、溶血的实验室证据、裂红细胞和血小板减少，随后出现伴有呼吸窘迫的急性脑病，符合TTP表现。发现她脑部有多处梗死灶。她接受了插管，并进行了血浆置换和红细胞置换。骨髓活检证实其血管闭塞性危象导致骨髓坏死，继而引发脂肪栓塞综合征。在此，我们讨论骨髓坏死所致脂肪栓塞的复杂表现及并发症，以及它如何酷似TTP。
