Clinical analysis of 59 patients with thrombotic thrombocytopenic purpura

Objective To investigate the clinical features, diagnosis, treatment and prognosis of 59 patients with thrombotic thrombocytopenic purpura (TTP), therefore to improve the ability of diagnosis and treatment. Methods The clinical data of 59 patients with TTP admitted to Peking University People's Hospital from January 2004 to October 2018 were retrospectively analyzed. All the patients were clinically diagnosed, fulfilled the triad syndrome, or quinary syndrome. Laboratory data included complete blood count, blood biochemistry, immunology, hemolysis; some patients tested the activity of ADAMTS13.The differences between groups were compared according to the prognosis. Results Among the 59 patients with TTP, 21 were male and 38 were female, with an average age of 46.8 years. Fifty-five patients had the triad syndrome and 46 patients had the quinary syndrome. The platelet count and hemoglobin decreased, the percentage of erythrocyte fragmented increased, and the value or the activity of ADAMTS13 was decreased significantly. PLASMIC scores of 57 patients were between 6 and 7. All 59 patients were treated with glucocorticoid, 41 patients received plasma exchange (PEX), and 28 patients survived; 18 patients did not received PEX, and only 6 patients survived. There was a significant difference of the survival between the two groups (P<0.05). Six patients were treated with rituximab and four patients survived. Conclusion The PLASMIC score can predict the activity of ADAMTS13 well. PEX can significantly improve the survival rate of patients with TTP. Key words: Thrombotic thrombocytopenic purpura; ADAMTS13; PLASMIC score; Plasma exchange; Prognosis