ABSTRACT
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 tilde 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