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San Carlo Canavese, Italy

Lotta L.A.,University of Milan | Mariani M.,University of Milan | Consonni D.,Unit of Epidemiology | Mancini I.,University of Milan | And 6 more authors.
British Journal of Haematology | Year: 2010

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:), 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. © 2010 Blackwell Publishing Ltd.

Benevolo G.,Haematology 2 Azienda Ospedaliera Cittadella Salute e della Science | Stacchini A.,University of Turin | Spina M.,Italian National Cancer Institute | Ferreri A.J.M.,San Raffaele Scientific Institute | And 16 more authors.
Blood | Year: 2012

This prospective study compared diagnostic and prognostic value of conventional cytologic (CC) examination and flow cytometry (FCM) of baseline samples of cerebrospinal fluid (CSF) in 174 patients with newly diagnosed aggressive non-Hodgkin lymphoma (NHL). FCM detected a neoplastic population in the CSF of 18 of 174 patients (10%), CC only in 7 (4%; P <.001); 11 patients (14%) were discordant (FCM+/CC-). At a median follow-up of 46 months, there were 64 systemic progressions and 10 CNS relapses, including 2 patients with both systemic and CNS relapses. Two-year progression-free and overall survival were significantly higher in patients with FCM- CSF (62% and 72%) compared with those FCM+ CSF (39% and 50%, respectively), with a 2-year CNS relapse cumulative incidence of 3% (95% confidence interval [CI], 0-7) versus 17% (95% CI, 0-34; P =.004), respectively. The risk of CNS progression was significantly higher in FMC+/CC - versus FCM-/CC- patients (hazard ratio = 8.16, 95% CI, 1.45-46). In conclusion, FCM positivity in the CSF of patients with high-risk NHL is associated with a significantly higher CNS relapse risk and poorer outcome. The combination of IV drugs with a higher CNS bioavailability and intrathecal chemotherapy is advisable to prevent CNS relapses in FCM+ patients. © 2012 by The American Society of Hematology.

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