Diagnosis and management of acquired thrombotic thrombocytopenic purpura in southeast China: a single center experience of 60 cases
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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.
Keywordsthrombotic thrombocytopenic purpura ADAMTS 13 plasma exchange
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- 3.Sadler JE, Moake JL, Miyata T, George JN. Recent advances in thrombotic thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program 2004; 2004(1): 1–407Google Scholar
- 4.Thrombosis, Hemostasis group HSCMA. Consensus of Chinese experts on diagnosis and treatment of thrombotic thrombocytopenic purpura (version 2012). Chin J Hematol (Zhonghua Xue Ye Xue Za Zhi) 2012; 33(11): 11–983(in Chinese)Google Scholar
- 5.Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ; British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol 2012; 158(3): 3–323CrossRefGoogle Scholar
- 6.Gao WQ, Su J, Bai X, Wang ZY, Ruan CG. Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura. Chin Med J (Engl) 2004; 117(6): 6–818Google Scholar
- 17.Blombery P, Kivivali L, Pepperell D, Mc Quilten Z, Engelbrecht S, Polizzotto MN, Phillips LE, Wood E, Cohney S; TTP registry steering committee. Diagnosis and management of thrombotic thrombocytopenic purpura (TTP) in Australia: findings from the first 5 years of the Australian TTP/thrombotic microangiopathy registry. Intern Med J 2016; 46(1): 1–71CrossRefGoogle Scholar
- 23.Plaimauer B, Kremer Hovinga JA, Juno C, Wolfsegger MJ, Skalicky S, Schmidt M, Grillberger L, Hasslacher M, Knöbl P, Ehrlich H, Scheiflinger F. Recombinant ADAMTS13 normalizes von Willebrand factor-cleaving activity in plasma of acquired TTP patients by overriding inhibitory antibodies. J Thromb Haemost 2011; 9(5): 5–936CrossRefGoogle Scholar