Prognostic factors in thrombotic thrombocytopenic purpura

Abstract

Abstract Objectives Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy with no standardized prognostic model to predict mortality. The aim of the study is to determine parameters associated with TTP-related mortality. Methods In this cross-sectional, retrospective study, 77 TTP patients, treated with therapeutic plasma exchange between 2001 and 2019 in Ankara University Faculty of Medicine were included. Results There was no significant relationship between ADAMTS13 inhibitor levels, activity and mortality. Median number of plasmapheresis was 10 (2–32), higher in patients with complete response. Anemia, kidney injury and LDH levels were associated with survival; there was no significant relationship between platelet counts at the time of diagnosis and mortality. Mortality was lower in patients with platelet counts above 100 × 109/L and normalized LDH after treatment. Hemoglobin, albumin, LDH and creatinine levels at the time platelet counts exceeded 50 × 109/L were associated with survival. Conclusions We determined several clinical and laboratory parameters associated with mortality. Fewer numbers of plasmapheresis was associated with mortality; thus other treatments as rituximab and caplacizumab should be considered early in non-responders. Including changes in laboratory parameters may be considered in prognostic scoring systems to be developed in the future.