Abstract
Abstract
Background
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.
Case presentation
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%.
Conclusions
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.
Funder
China Postdoctoral Science Foundation
Science and Technology Project of Nantong City
Publisher
Springer Science and Business Media LLC
Subject
Obstetrics and Gynecology
Reference34 articles.
1. Plautz W, Raval J, Dyer M, Rollins-Raval M. ADAMTS13: origins, applications, and prospects. Transfusion. 2018;58(10):2453–62.
2. Scully M, Cataland S, Coppo P. J dlR, KD F, J KH. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017;15(2):312–22.
3. Shah AA, Higgins JP, Chakravarty EF. Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties. Nature. 2007;3(6):357–62.
4. Joly B, Coppo P, Veyradier A. Thrombotic thrombocytopenic purpura. Blood. 2017;129(21):2836–46.
5. Vesely SK, Li X, McMinn JR, Terrell DR, George JN. Pregnancy outcomes after recovery from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Transfusion. 2004;44(8):1149–58.
Cited by
4 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献