1. Application of the French <scp>TMA</scp> Reference Center Score and the mortality in <scp>TTP Score</scp> in de novo and relapsed episodes of acquired <scp>thrombotic thrombocytopenic purpura</scp> at a tertiary care facility in Spain
- Author
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Gloria Pérez-Rus, Isabel Regalado-Artamendi, Ana Pérez-Corral, Amalia Domingo-González, José Luis Díez-Martín, Reyes María Martín-Rojas, and Cristina Pascual-Izquierdo
- Subjects
medicine.medical_specialty ,Acquired Thrombotic Thrombocytopenic Purpura ,business.industry ,Mortality rate ,Significant difference ,Clinical course ,Hematology ,General Medicine ,030204 cardiovascular system & hematology ,Tertiary care ,Adamts13 activity ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Medicine ,business ,030215 immunology - 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
- Published
- 2021
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