"Background: Thrombotic thrombocytopenic purpura is a relatively rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage. The early recognition is critical because it is an emergency condition requiring urgent therapeutic plasma exchange, which is a life-saving procedure and the cornerstone of the management.
Methods: A retrospective descriptive study was conducted at the Department of Medicine-Hematology at King Hussein Medical Center, Amman, Jordan, between June 2018 and July 2019. All patients from all departments who were diagnosed with Thrombotic thrombocytopenic purpura during the study period were enrolled. We obtained the information from all patients concerning demographic data, signs, and symptoms of presentation, and the results of investigations at admission and discharge. The number of plasma exchange sessions, days of hospitalization, usage of Rituximab, and outcome.
Results: 21 patients were enrolled, predominantly female ( 67%), the median age was 36.67 years. At diagnosis, 81% of patients showed neurological involvement. The median platelet count at presentation was 20.000/μL. All patients were anemic (mean HCT,25.42). Schistocytes were present in the peripheral blood film of all patients. The median number of plasma exchange sessions was 19 (range, 242), and the mean stay in hospital was 25 ± 11.6 days. Rituximab was administered to 11 patients (52%). A complete response was achieved in 62% (13/21) of patients, The mortality rate was 38% (8/21 patients). None of the patients characteristics or laboratory results at presentation were significantly associated with mortality.
Conclusions: Although our study included a limited number of TTP patients, our findings revealed that our patients had poor prognostic factors and were less responsive to PEX than TTP patients in other countries. Our data also confirm a better survival rate in younger patients than in the elderly.
Key words: Thrombotic Thrombocytopenic Purpura, Jordan, Plasma Exchange, Rituximab