Morrissey Dylan, Sun Yuheng, Koilpillai Sarina, Kropf Jacqueline, Carlan Steve J
Department of Internal Medicine, Orlando Regional Healthcare, Orlando, Florida, USA.
Division of Academic Affairs and Research, Orlando Regional Healthcare, Orlando, Florida, USA.
Case Rep Med. 2022 Sep 3;2022:7306070. doi: 10.1155/2022/7306070. eCollection 2022.
Clinical B deficiency with hematological or neurological manifestations is rare. An unusual manifestation of B deficiency is pseudo-thrombotic microangiopathy (TMA), which is characterized by hemolytic anemia, thrombocytopenia, and schistocytosis and only occurs in 2.5% of those with B deficiency. Pseudo-TMA is misdiagnosed as thrombotic thrombocytopenic purpura (TTP) in 40% of cases, resulting in misguided treatment including plasmapheresis.
A 44-year-old Hispanic presented with 3 weeks of progressively worsening non-radiating chest pain, fatigue, and shortness of breath (SOB). Laboratory findings revealed severe pancytopenia and macrocytosis with a hemoglobin of 5.4 g/dL, mean corpuscular volume of 116.3 fL, and vitamin B low at 149 pg/mL. She was diagnosed with pseudo-TMA and after starting 1000 micrograms of parenteral vitamin B injections daily and discontinuing plasmapheresis and steroid administration, she improved.
Failure to recognize pseudo-TMA often results in unnecessary treatment with plasmapheresis and delays appropriate treatment with vitamin B supplementation. It is therefore extremely important to consider pseudo-TMA as a differential diagnosis in patients that present with hemolytic anemia, thrombocytopenia, and schistocytosis.
伴有血液学或神经学表现的临床维生素B缺乏症较为罕见。维生素B缺乏症的一种不寻常表现是假性血栓性微血管病（TMA），其特征为溶血性贫血、血小板减少和裂体细胞增多，仅在2.5%的维生素B缺乏患者中出现。40%的假性TMA病例被误诊为血栓性血小板减少性紫癜（TTP），导致包括血浆置换在内的治疗不当。
一名44岁的西班牙裔患者出现3周来逐渐加重的非放射性胸痛、疲劳和呼吸急促（SOB）。实验室检查发现严重全血细胞减少和大细胞性贫血，血红蛋白为5.4g/dL，平均红细胞体积为116.3fL，维生素B水平低至149pg/mL。她被诊断为假性TMA，在开始每日注射1000微克肠胃外维生素B并停止血浆置换和类固醇给药后，病情有所改善。
未能识别假性TMA常常导致不必要的血浆置换治疗，并延误补充维生素B的适当治疗。因此，对于出现溶血性贫血、血小板减少和裂体细胞增多的患者，将假性TMA作为鉴别诊断极其重要。
