Different clinical severity of first episodes and recurrences of thrombotic thrombocytopenic purpura.
The clinical course of thrombotic thrombocytopenic purpura (TTP) is characterized by recurrent disease episodes in up to 50% of 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, derived 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.
Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Luigi Villa Foundation, Department of Medicine and Medical Specialities, Università degli Studi di Milano, Milan, Italy.
Luca A
LA
Lotta
Mariagabriella
M
Mariani
Dario
D
Consonni
Ilaria
I
Mancini
Roberta
R
Palla
Alberto
A
Maino
Dragica
D
Vucelic
Michele
M
Pizzuti
Pier M
PM
Mannucci
Flora
F
Peyvandi
D051722:ADAM Proteins / Q000172:deficiency; D000071120:ADAMTS13 Protein; D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000453:epidemiology / Q000209:etiology; D010976:Platelet Count; D011379:Prognosis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D012008:Recurrence; D012042:Registries
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human