Adult idiopathic thrombotic thrombocytopenic purpura therapeutic modalities and outcome: 15 years’ experience

Background Thrombotic thrombocytopenic purpura (TTP) is a rare hematological disease that results in fatality if not treated. The studies on TTP are scarce; most of them have small numbers of patients. Since not enough data on Egyptian patients with TTP can be found in the literature, we carried out this retrospective study to define the therapeutic modalities and outcome of these patients. Patients and methods We studied the records of patients clinically diagnosed with TTP from 2006 to 2020. Twenty-nine patients were diagnosed with TTP. However, full records were available only for 22 patients. The primary outcome of the study was the 1-year survival. Other outcomes were the factors on which the survival seemed to depend on such as age, platelet count, lactate dehydrogenase (LDH), serum creatinine, hemoglobin, reticulocytic %, plasma volume exchange (PVE), days to start of treatment, and indirect bilirubin. Results Platelet count change was negatively correlated with both the number of plasma exchange sessions and the days to start of treatment, but it was positively correlated with the volume of plasma exchange. Univariate analysis identified factors associated with better survival of the patients: LDH, reticulocytic percent, days to start of treatment, serum creatinine, PVE, and hemoglobin level. The multivariate analysis revealed that the independent factors affecting the survival were LDH, reticulocytic percent, hemoglobin level, and age. Conclusion Early suspicion and intervention with therapeutic plasma exchange for TTP are associated with better outcomes in these patients. One and a half PVE is associated with better response than 1 PVE.