血栓性血小板减少性紫癜83例临床分析

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.