Clinical characteristics of 83 patients with thrombotic thrombocytopenic purpura.
10.3760/cma.j.issn.0253-2727.2020.03.006
- VernacularTitle:血栓性血小板减少性紫癜83例临床分析
- Author:
Xi Yan WANG
1
;
Xiao Fan LIU
1
;
Feng XUE
1
;
Wei LIU
1
;
Yun Fei CHEN
1
;
Yue Ting HUANG
1
;
Rong Feng FU
1
;
Lei ZHANG
1
;
Ren Chi YANG
1
Author Information
1. State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China.
- Publication Type:Journal Article
- Keywords:
PLASMIC score;
Thrombotic thrombocytopenic purpura;
von Willebrand factor cleaving protease 13
- MeSH:
ADAM Proteins;
ADAMTS13 Protein;
Adolescent;
Adult;
Aged;
Child;
Female;
Humans;
Male;
Middle Aged;
Plasma Exchange;
Purpura, Thrombotic Thrombocytopenic;
Retrospective Studies;
Rituximab;
Young Adult
- From:
Chinese Journal of Hematology
2020;41(3):216-221
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To analyze the clinical characteristics, treatment and prognosis of patients with thrombotic thrombocytopenic purpura (TTP) . Methods: 83 patients with TTP from May 1998 to May 2019 were analyzed retrospectively. Results: Among the 83 patients, there were 27 males and 56 females, with a median age of 39 (10-68) years. 41 cases (49.4%) showed pentalogy syndrome and 79 cases (95.2%) showed triad syndrome. 78.0% (46/59) of the patients had a PLASMIC score of 6 or higher. TTP gene mutations was detected in 5 of 10 patients. The activity of von Willebrand factor-cleaving protease (ADAMTS13) , which was detected in 10 patients before plasma exchange (PEX) , was less than 10% in 9 patients. 83 patients were treated with PEX/plasma infusion and glucocorticoid, 35 of which were treated combined with rituximab and/or immunosuppressant. The median follow-up was 34 (1-167) months, the effective rate was 81.9%, the remission rate was 63.9%, the relapse rate was (35.7 ±7.1) %, and the 3-year overall survival (OS) rate was (78.6 ±4.6) %. The effective rate (72.9%vs 94.3%, P=0.019) and OS rate[ (63.8±7.5) %vs (94.3±3.9) %, χ(2)=8.450, P=0.004] in the group treated with PEX/PI and glucocorticoid alone were lower than those in the group treated combined with rituximab and/or immunosuppressant. COX multivariate analysis showed that age (HR=1.111, 95%CI 1.044-1.184, P=0.001) and alanine transaminase (ALT) /aspartate aminotransferase (AST) (HR=1.353, 95%CI 1.072-1.708, P=0.011) were independent risk factors for OS. Conclusion: Most patients with TTP have triad syndrome, accompanied by a decrease in ADAMTS13 activity. Plasma infusion and glucocorticoid combined with rituximab, immunosuppressive therapy could improve overall survival. The prognosis of patients with older age and high ALT/AST ratio is poor.
