Clinical Features and Prognosis of Thrombotic Thrombocytopenic Purpura

  Objective   The aim of this study was to explore the clinical characteristics and prognosis of thrombotic thrombocytopenic purpura (TTP).

  Methods   Clinical manifestations, laboratory examinations, diagnosis, therapeutic methods, and prognosis of 60 TTP patients diagnosed between 2012 and 2017 in Peking Union Medical College Hospital were retrospectively analyzed. Risk factors of prognosis were analyzed by single factor and multivariate Logistic regression analysis, and the influence of therapeutic strategieson prognosis was analyzed by Kruskal-Wallis test and Mann-Whitney U test.

  Results   Among 60 TTP patients, 17 were males (28.3%, 17/60) and 43 were females (71.7%, 43/60), aged (41±15) years old. Twenty-eight patients (46.7%, 28/60) had Triad Syndrome including fever, microangiopathic hemolytic anemia, and thrombocytopenia; 23 patients (38.3%, 23/60) had Quinary Syndrome including fever, microangiopathic hemolytic anemia, thrombocy-topenia, renal insufficiency, and neurological symptoms; and the other 9 patients (15.0%, 9/60) have neither the typical Triad Syndrome nor the Quinary Syndrome. Twenty-eight patients received the measurement for ADAMTS13 activity and 23 (82.1%, 23/28) was < 10% among whom; ADAMTS13 inhibitor was tested in 20 patients with 18 (90.0%, 18/20) of them being positive; 20 patients received these both tests and the double positive rate was 90% (18/20). Different treatments, which included plasma exchange, glucocorticoids, rituximab, immunosuppresors, and intravenous immunoglobulin, were given based on patients' financial situation and blood fountain; 42 patients (70.0%, 42/60) were relieved and 18 patients died (30.0%, 18/60). The combined immunosuppressive therapy on the basis of plasma exchange and glucocorticoids improved the remission rate from 57.1% to 85.2% (P=0.032). No risk factor was found between the dead group and the remission group.

  Conclusions   Most of TTP patients manifest the Triad Syndrome or the Quinary Syndrome. The decline of ADAMTS13 activity and its inhibitor have high positive rates in TTP. Immunosuppressive therapy can improve the prognosis.