Thrombotic thrombocytopenic purpura - Analysis of clinical features,laboratory characteristics and therapeutic outcome of 24 patients treated at a tertiary care center in Saudi Arabia

Objective: thrombotic thrombocytopenic purpura (ttp) is a life-threatening disease. the primary aim was overall response rate (orr) assessment in the treated patients. methods: this retrospective study included 24 patients treated during 2006-2015. ttp patients with microangiopathic hemolysis (maha) and thrombocytopenia were included. we analyzed clinical features,laboratory characteristics and treatment outcomes of 24 ttp patients treated at our tertiary care center (kfmc). results: twenty-four ttp patients (18 females; 6 males) had a mean age of 33.5±13.9 years; 22(91%) had neurologic features,7(29%) fever,10(42%) renal impairment; 4(20.83%) cardiac manifestations; 22(91.7%) had triad with additional neurologic abnormalities; only 2(8.2%) had pentad of ttp. majority (54.16%) had idiopathic ttp. all patients received therapeutic plasma exchange (tpe); 23(95.8%) received adjunctive corticosteroids and 13(54.2%) received rituximab either due to refractoriness to tpe on ~ day7,or earlier. twenty-one out of 24 (87.5%) achieved complete remission (cr) without any subsequent relapse. at 22 months (median,range 1-113),20 patients (83.3%) are alive at the time of report. three patients died during acute episode because of sever disease or delayed treatment and one died in cr. conclusion: tpe,steroids and or rituximab was very effective in preventing high risk of mortality and achieving durable cr in 87.5% of patients. more awareness is needed for early diagnosis and early referral to centers with appropriate tertiary care facilities. © 2016,professional medical publications. all rights reserved.