title: 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.
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Xinping
authors.initials: X
authors.lastname: Zhou
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.|Department of Hematology, the Forth Affiliated Hospital, College of Medicine, Zhejiang University, Yiwu, 322000, China.
authors.forename: Xingnong
authors.initials: X
authors.lastname: Ye
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Yanling
authors.initials: Y
authors.lastname: Ren
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Chen
authors.initials: C
authors.lastname: Mei
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Liya
authors.initials: L
authors.lastname: Ma
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Jiansong
authors.initials: J
authors.lastname: Huang
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Weilai
authors.initials: W
authors.lastname: Xu
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Juying
authors.initials: J
authors.lastname: Wei
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Li
authors.initials: L
authors.lastname: Ye
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Wenyuan
authors.initials: W
authors.lastname: Mai
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Wenbin
authors.initials: W
authors.lastname: Qian
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Haitao
authors.initials: H
authors.lastname: Meng
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China.
authors.forename: Jie
authors.initials: J
authors.lastname: Jin
authors.affiliation: Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, 310003, China. hongyantong@aliyun.com.
authors.forename: Hongyan
authors.initials: H
authors.lastname: Tong
mesh_terms: D000071120:ADAMTS13 Protein / Q000276:immunology; D000293:Adolescent; D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal / Q000097:blood; D002681:China; D016572:Cyclosporine / Q000627:therapeutic use; D019468:Disease Management; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy*; D012008:Recurrence; D000069283:Rituximab / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000911:Antibodies, Monoclonal; D007166:Immunosuppressive Agents; D000069283:Rituximab; D014750:Vincristine; D016572:Cyclosporine; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS 13; plasma exchange; thrombotic thrombocytopenic purpura