[Analysis of Prognosis and Therapeutic Effect of Patients with Thrombotic Thrombocytopenic Purpura].

OBJECTIVE: To summarize the features of clinical and laboratory parameters of thrombotic thrombocytopenic purpura (TTP), and to analyze the factors affecting the prognosis and therapeutic effect during the acute phase of the disease.

METHODS: The etiology, clinical features, laboratory parameters, treatment regimens and other data of 59 TTP patients admitted to Hunan Provincial People's Hospital were retrospectively analyzed. And the differences of each variable between the death group and the survival group were compared, the correlations between each variable and prognosis, as well as the therapeutic effect of the acute phase patients were analyzed.

RESULTS: Among the 59 cases of TTP, one 4-year-old boy was inherited TTP, the other 58 cases were acquired TTP (39 cases were idiopathic TTP and 19 were secondary TTP), including 27 males and 31 females, with a median age of 54 (11-84) years old. 36 patients were tested for von Willebrand factor cleaving protease (ADAMTS13) activity, and 34 patients (94.44%) had decreased ADAMTS13 activity. There was a statistical difference in the activity of ADAMTS13 between idiopathic and secondary TTP patients ( P <0.001). There were statistical differences in factors such as age (64 vs 51 years), ALT/AST ratio (0.61 vs 0.36), therapeutic plasma exchange (TPE) times (3 times vs 8 times), and TPE duration (3 d vs 9 d) between the death and survival groups. Multivariate analysis showed that total times of TPE ( OR =5.175,95% CI : 1.169-22.914, P =0.030), ALT/AST ratio ( OR =4.387, 95% CI : 1.019-18.891, P =0.047) were associated with mortality. COX regression analysis showed that degree of neuropsychiatric disorder ( HR =0.200, 95% CI : 0.084-0.474, P <0.001), days from onset to initiation of TPE ( HR =0.288, 95% CI : 0.114-0.726, P =0.008), treatment regimen ( HR =0.336, 95% CI : 0.125-0.902, P =0.030) were associated with platelet recovery and therapeutic effect in the acute phase of the patients.

CONCLUSION: TTP patients with high ALT/AST ratio have a higher mortality rate. Patients with enough times and full course of TPE have lower mortality rate. Poor therapeutic effect were found in TTP patients with severe neuropsychiatric disorders, delayed diagnosis and treatment, and who added rituximab when TPE was ineffective.