Diagnosis and treatment of thrombotic thrombocytopenic purpura: A single institution experience

Background: Thrombotic thrombocytopenic purpura (TTP) is an urgent, life-threatening hematologic disorder. It can result in a high fatality rate without early identification and prompt plasma exchange (PE) therapy. We report our experience of clinical manifestations, laboratory abnormalities, diagnosis, treatment and outcome of patients with TTP at our institution. Patients and Methods: Medical records of 13 patients diagnosed with TTP at our institution from 1997 to 2009 were reviewed. Relevant information including age, gender, clinical presentation, predisposing factors, laboratory abnormalities, treatment, complications, and outcome of each patient were analyzed. Results: The median age of the patients was 47 years with a range of 14-78. Five of the 13 (38%) patients presented with the full "pentad" of all clinical features. The median hemoglobin and platelet count at diagnosis was 8.3 g/dL and 9×10 ³/μL, respectively. The median time from onset of symptoms to diagnosis was 14 days. Nine of the 13 patients (70%) had the sporadic or idiopathic form of TTP. The other cases of TTP were associated with ticlopidine, breast cancer and autoimmune diseases. The median number of PE was 11 (range: 6-37). Nine of 13 patients (70%) were treated successfully with the combination of PE and corticosteroids, and the remaining 4 patients who failed to respond to this therapy eventually died of multi-organ failure. Conclusion: Our study showed 38% of such patients presented with the full pentad of clinical features but the median time from symptom onset to diagnosis and initiation of therapy was 14 days for the entire cohort. Treatment with a combination of corticosteroids and PE resulted in a 70% success rate in achieving long term remission.