DIFFERENT CLINICAL SEVERITY OF FIRST EPISODES AND RECURRENCES OF THROMBOTIC THROMBOCYTOPENIC PURPURA.

Luca Andrea Lotta (1) , Mariagabriella Mariani (2) , Dario Consonni (3) , Ilaria Mancini (2) , Roberta Palla (2) , Alberto Maino (2) , Dragica Vucelic (4) , Michele Pizzuti (5) , Piermannuccio Mannucci (2) , Flora Peyvandi (2)

1 Department of Medicine and Medical Specialties
2 Department of Medicine and Medical Specialties
3 Unit of Epidemiology
4 Institute of Gastrointestinal Diseases
5 Hematology division

The clinical course of thrombotic thrombocytopenic purpura (TTP) is characterized by recurrent disease episodes in up to 50% of the cases. The clinical presentation and severity of different TTP episodes have not been systematically compared. Laboratory and clinical information from 51 patients with recurrent disease, stemming from 136 patients with TTP included in the Milan TTP registry (URL: http://www.ttpdatabase.org), were used to compare mortality, symptoms and disease-related laboratory measurements in different disease episodes. The prevalence of severe neurological symptoms (coma, seizures and focal neurological defects) was significantly lower in recurrences than in the first episode. Platelet counts and haemoglobin levels at presentation were higher in recurrences than in the first disease episode, and lactate dehydrogenase levels were lower. Also mortality tended to be lower in the second and third disease episodes than in the first. Recurrences of TTP are generally milder than first episodes. These differences in severity should be taken into account in clinical research on TTP and in patient management.

Mots clés: Medicine

Luca Andrea Lotta, Mariagabriella Mariani, Dario Consonni, Ilaria Mancini, Roberta Palla, et al.. DIFFERENT CLINICAL SEVERITY OF FIRST EPISODES AND RECURRENCES OF THROMBOTIC THROMBOCYTOPENIC PURPURA.. British Journal of Haematology, 2010, 151 (5), pp.488. ⟨10.1111/j.1365-2141.2010.08385.x⟩. ⟨hal-00586988⟩