Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report
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...
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2021
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oai:doaj.org-article:383d3ed022914560bd92fe386737aaa32021-12-05T12:20:45ZPregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report10.1186/s12884-021-04167-91471-2393https://doaj.org/article/383d3ed022914560bd92fe386737aaa32021-12-01T00:00:00Zhttps://doi.org/10.1186/s12884-021-04167-9https://doaj.org/toc/1471-2393Abstract 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.Lu ZhouYu ZhuMiao JiangJian SuXiaofan LiuYizhi JiangHui MuJie YinLi YangHaiyan LiuWeidong PanMin SuHong LiuBMCarticleThrombotic thrombocytopenic purpuraPregnancyRituximabSjögren’s syndromeGynecology and obstetricsRG1-991ENBMC Pregnancy and Childbirth, Vol 21, Iss 1, Pp 1-6 (2021) |
| institution |
DOAJ |
| collection |
DOAJ |
| language |
EN |
| topic |
Thrombotic thrombocytopenic purpura Pregnancy Rituximab Sjögren’s syndrome Gynecology and obstetrics RG1-991 |
| spellingShingle |
Thrombotic thrombocytopenic purpura Pregnancy Rituximab Sjögren’s syndrome Gynecology and obstetrics RG1-991 Lu Zhou Yu Zhu Miao Jiang Jian Su Xiaofan Liu Yizhi Jiang Hui Mu Jie Yin Li Yang Haiyan Liu Weidong Pan Min Su Hong Liu Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report |
| description |
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. |
| format |
article |
| author |
Lu Zhou Yu Zhu Miao Jiang Jian Su Xiaofan Liu Yizhi Jiang Hui Mu Jie Yin Li Yang Haiyan Liu Weidong Pan Min Su Hong Liu |
| author_facet |
Lu Zhou Yu Zhu Miao Jiang Jian Su Xiaofan Liu Yizhi Jiang Hui Mu Jie Yin Li Yang Haiyan Liu Weidong Pan Min Su Hong Liu |
| author_sort |
Lu Zhou |
| title |
Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report |
| title_short |
Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report |
| title_full |
Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report |
| title_fullStr |
Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report |
| title_full_unstemmed |
Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report |
| title_sort |
pregnancy-associated thrombotic thrombocytopenic purpura complicated by sjögren’s syndrome and non-neutralising antibodies to adamts13: a case report |
| publisher |
BMC |
| publishDate |
2021 |
| url |
https://doaj.org/article/383d3ed022914560bd92fe386737aaa3 |
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