Diagnosis and management of acquired thrombotic thrombocytopenic purpura in southeast China: a single center experience of 60 cases

Abstract

Acquired thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy. This study aimed to provide a profile of the diagnosis and management of patients with acquired TTP collected in 10 years in a single center in southeast China. A total of 60 patients diagnosed with acute acquired TTP from March 2005 to August 2015 were enrolled. Among the 60 patients, 52 patients presented with their first episodes, and eight patients had two or more episodes. The median age at presentation was 49 (range, 17 to 78) years with a female predominance (male:female ratio, 1:1.60). ADAMTS 13 activity were analyzed in 43 patients, among whom 33 (76.7%) patients had a baseline level of<5%. Mortality was 30%. Plasma exchange (PEX) was performed in 62 of 69 (89.9%) episodes. Corticosteroids were administered in 54 of 69 (78.3%) episodes. Other immunosuppressants (e.g., vincristine, cyclosporine, and cyclosporin) were used in 7 of 69 (10.1%) episodes. Rituximab was documented in 4 patients with refractory/relapsed TTP for 5 episodes, showing encouraging results. In conclusion, the diagnosis of TTP depended on a comprehensive analysis of clinical data. Plasma ADAMTS13 activity assay helped confirm a diagnosis. PEX was the mainstay of the therapy, and rituximab can be used in relapsed/refractory disease.

Keywords

thrombotic thrombocytopenic purpura / ADAMTS 13 / plasma exchange

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Acknowledgements

We are thankful to all the laboratory members of Institute of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University for helpful discussion.

Compliance with ethics guidelines

Xinping Zhou, Xingnong Ye, Yanling Ren, Chen Mei, Liya Ma, Jiansong Huang, Weilai Xu, Juying Wei, Li Ye, Wenyuan Mai, Wenbin Qian, Haitao Meng, Jie Jin, and Hongyan Tong declare that they have no conflicts of interest.All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with theHelsinki Declaration of 1975, as revised in 2000 (5). Informed consent was obtained from all patients for being included in the study.

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2016 Higher Education Press and Springer-Verlag Berlin Heidelberg