血栓性血小板减少性紫癜(TTP)是一种罕见的妊娠并发症,其特征是微血管病性溶血性贫血和消耗性血小板减少症。我们在此描述了一名32岁女性的病例报告,该女性怀孕六周,患有双胞胎,并发展为血栓性血栓性紫癜(TTP)。病人有镰状细胞特征病史,偏头痛,和先兆子痫.她抱怨恶心,疲劳,喉咙痛,和咳嗽,发现贫血,血红蛋白为7g/dl,血小板减少,血小板计数为8x103/μL。患者迅速开始使用类固醇和血浆置换,具有出色的初始反应。然而,三天后,她突然出现头痛和呼吸急促,重复实验室显示贫血恶化(7.3g/dl)和血小板减少症(8x103/μL)。ADAMTS13活性显著低到2%。继续进行血浆置换,开始卡普拉斯单抗和利妥昔单抗治疗.胎儿超声显示胎儿两极没有心脏活动,患者因错过流产而进行了扩张和刮宫(D&C)。她用泼尼松锥度出院了,每日卡普拉珠单抗,和每周一次的利妥昔单抗.此病例报告强调了早期及时识别TTP的重要性,以及难治性TTP(rTTP)患者的适当管理策略,包括血浆置换,caplacizumab,还有利妥昔单抗.
Thrombotic thrombocytopenic purpura (TTP) is a rare pregnancy complication characterized by microangiopathic hemolytic anemia and consumption thrombocytopenia. We herein describe the case report of a 32-year-old woman who was six weeks pregnant with twins and developed thrombotic thrombocytic purpura (TTP). The patient had a history of sickle cell trait, migraines, and preeclampsia. She presented with complaints of nausea, fatigue, sore throat, and cough and was found to be anemic with a hemoglobin of 7 g/dl and thrombocytopenic with a platelet count of 8 x 103/μL. The patient was promptly initiated on steroids and plasmapheresis with an excellent initial response. However, after three days, she developed a sudden onset headache and shortness of breath, and repeat labs showed worsening anemia (7.3 g/dl) and thrombocytopenia (8 x 103/μL). ADAMTS13 activity was significantly low at 2%. Plasmapheresis was continued, and caplacizumab and rituximab treatment was initiated. The fetal ultrasound showed no cardiac activity in the fetal poles, and the patient had a dilation and curettage (D&C) for a missed abortion. She was discharged with a prednisone taper, daily caplacizumab, and weekly rituximab. This case report underscores the criticality of the prompt identification of TTP in its early stages, and appropriate management strategies for patients with refractory TTP (rTTP), including plasmapheresis, caplacizumab, and rituximab.