Hematology department, Affiliated Hospital of Nantong University, No 20 Xisi Road, Nantong, 226001, Jiangsu, China.
Obstetrics and Gynecology Department, Affiliated Hospital of Nantong University, No 20 Xisi Road, Nantong, 226001, Jiangsu, China.
BMC Pregnancy Childbirth. 2021 Dec 3;21(1):804. doi: 10.1186/s12884-021-04167-9.
Thrombotic thrombocytopenic purpura (TTP) is a severe and life-threatening disease. Given its heterogeneous clinical presentation, the phenotype of TTP during pregnancy and its management have not been well documented.
We report here a 25-year-old woman, G1P0 at 36 weeks gestation, who developed severe thrombocytopenia and anemia. She was performed an emergent caesarean section 1 day after admission because of multiple organ failure. As ADAMTS 13 enzyme activity of the patient was 0% and antibodies were identified by enzyme-linked immunosorbent assay, she was diagnosed as acquired thrombotic thrombocytopenic purpura (aTTP). Furthermore, asymptomatic primary Sjögren's syndrome was incidentally diagnosed on screening. After treatment with rituximab in addition to PEX and steroids, the activity of the ADAMTS 13 enzyme increased significantly from 0 to 100%.
To the best of our knowledge, this is the first case report of concomitant TTP and asymptomatic Sjögren's syndrome in a pregnant woman. It highlights the association between pregnancy, autoimmune disease, and TTP. It also emphasizes the importance of an enzyme-linked immunosorbent assay in the diagnosis and rituximab in the treatment of patients with acquired TTP.
血栓性血小板减少性紫癜（TTP）是一种严重且危及生命的疾病。鉴于其临床表现的异质性，妊娠期间 TTP 的表型及其管理尚未得到很好的记录。
我们在此报告一例 25 岁、G1P0 孕 36 周的患者，因严重血小板减少和贫血入院。入院后 1 天因多器官功能衰竭行急诊剖宫产术。由于患者 ADAMTS13 酶活性为 0%，且通过酶联免疫吸附试验检测到抗体，故诊断为获得性血栓性血小板减少性紫癜（aTTP）。此外，在筛查时还意外诊断出无症状原发性干燥综合征。在接受利妥昔单抗联合 PEX 和类固醇治疗后，ADAMTS13 酶的活性从 0 显著增加至 100%。
据我们所知，这是首例妊娠合并 TTP 和无症状干燥综合征的病例报告。该病例强调了妊娠、自身免疫性疾病和 TTP 之间的关联。还强调了酶联免疫吸附试验在诊断和利妥昔单抗治疗获得性 TTP 中的重要性。
