Application of the French TMA Reference Center Score and the mortality in TTP Score in de novo and relapsed episodes of acquired thrombotic thrombocytopenic purpura at a tertiary care facility in Spain

Abstract

Acquired thrombotic thrombocytopenic purpura (aTTP) is still associated with a 10% to 20% death rate and its clinical course is characterized by recurrent episodes in up to 50% of cases. Over the last decade, mortality predicting models like the French TMA Reference Center Score and the Mortality In TTP Score (MITS) have been developed in an attempt to personalize treatment. The objective of the present study was to compare the results in both scores of de novo and relapsed aTTP episodes. For such purpose, a total of 29 episodes of aTTP (16 de novo and 13 relapses) were analyzed. All patients were homogeneously diagnosed and treated. First episodes had a higher score in both models in comparison with relapsed aTTP, (MITS median, 1 r: 1‐4 vs 0 r: 1‐2, P = .038 and French TMA Reference Center Score median, 2 r: 1‐3 vs 1 r: 0‐1, P = .006). The prevalence of neurological symptoms was significantly higher in the first episodes (P = .001) and patients >60 years old were more common in this group (P = .013), which may have been related to the results. Platelet count at presentation was higher in recurrences than in the first disease episode (P = .016) and ADAMTS13 activity <5% was more frequent in the last group (P = .016). There was no significant difference in the rate of refractoriness or exacerbations. In conclusion, first aTTP episodes had a higher probability of short‐term mortality compared to relapsed aTTP episodes according to the MITS and French TMA Reference Center Score.

Effectiveness of Caplacizumab Nanobody in Acquired Thrombotic Thrombocytopenic Purpura Refractory to Conventional Treatment

“…In our case report, we observed relevant reductions in time to platelet count restoration (4 vs. 7 days), days of PEX (4 vs. 8 days) and duration of hospitalization (4 vs. 9 days) comparing the previous recurrence with standard therapy and the last relapse adding caplacizumab. Some reports have suggested using ADAMTS13 levels to predict aTTP relapse [9,10]. Unfortunately, the measurement of ADAMTS13 was not available in our centre until 2018, and this marker was not employed in the first cases of recurrence.…”

Immune and Hereditary Thrombotic Thrombocytopenic Purpura: Can ADAMTS13 Deficiency Alone Explain the Different Clinical Phenotypes?

“…Despite adequate treatment, 10% of patients will die from this disease, and up to 50% of patients will have recurrent episodes [28]. A recent study was performed in Spain with the application of the French TMA Reference Center Score and the mortality in TTP Score in 20 patients suffering from de novo and relapsed episodes of iTTP [50]. The median age of these patients was 46 (IQR 39-56).…”