Elayoubi Jailan, Donthireddy Kavitha, Nemakayala Divyesh Reddy
Department of Hematology and Oncology, Michigan State University, Lansing, Michigan, USA.
Department of Hematology and Oncology, Howard Hughes Medical Institute, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
BMJ Case Rep. 2018 Jan 17;2018:bcr-2017-221648. doi: 10.1136/bcr-2017-221648.
Thrombotic thrombocytopenic purpura (TTP) is a potentially reversible, life-threatening medical emergency. We present a case of a 21-year-old female with evidence of haemolytic anaemia based on the presence of positive markers of haemolysis. Negative Coomb's test, thrombocytopenia and placental infarcts raised suspicion for a thrombotic microangiopathy. She was diagnosed with TTP and managed with emergency plasma exchange. Her recovery was immediate.A presumptive diagnosis of TTP should be based on the presence of microangiopathic haemolytic anaemia with thrombocytopenia and plasma exchange should be initiated while complete work up is pending. Using the regular pentad solely for diagnosis of TTP will lead to underdiagnosis of many cases and should be avoided.Several microangiopathies can be seen during pregnancy including TTP/atypical haemolytic uraemic syndrome, HELLP syndrome, pre-eclampsia, disseminated intravascular coagulopathy and antiphospholipid antibody syndrome. Distinction between each type will be the focus of our discussion as treatment decisions differ accordingly.
血栓性血小板减少性紫癜(TTP)是一种潜在可逆的、危及生命的医疗急症。我们报告一例21岁女性病例,基于溶血阳性标志物的存在,有溶血性贫血的证据。抗人球蛋白试验阴性、血小板减少和胎盘梗死增加了血栓性微血管病的怀疑。她被诊断为TTP并接受了紧急血浆置换治疗。她立即康复。TTP的初步诊断应基于微血管病性溶血性贫血伴血小板减少,在完成全面检查之前应启动血浆置换。仅使用经典五联征来诊断TTP会导致许多病例漏诊,应避免这种做法。妊娠期间可见多种微血管病,包括TTP/非典型溶血性尿毒症综合征、HELLP综合征、子痫前期、弥散性血管内凝血和抗磷脂抗体综合征。由于治疗决策相应不同,区分每种类型将是我们讨论的重点。