THE REGISTRO ITALIANO TROMBOCITEMIE (RIT): PRELIMINARY ANALYSIS OF THE FIRST 767 ENROLLED PATIENTS Results L. Gugliotta and A. Tieghi for the RIT Panel * Background RIT Institutions • Alessandria • Ancona •Ascoli Piceno •Avellino •Bari •Bologna •Bolzano •Brindisi •Cagliari •Campobasso •Carbonara di Bari •Castelfranco Veneto •Catania •Catanzaro •Civitanova Marche •Como •Cosenza •Crema •Cremona •Firenze •Firenze Ist.L.da Vinci •Genova S.Martino •Genova DIMI •Genova Ematologia I •L’Aquila •La Spezia •Mantova •Matera •Messina Papardo •Messina Policlinico •Milano S.Paolo •Milano Ist. Tumori •Milano Policlinico •Milano S. Raffaele •Milano Ematologia II •Modena •Montefiascone •Monza •Napoli S.Gennaro •Napoli S.G.Bosco •Napoli Federico II •Nocera Inferiore •Novara •Orbassano •Padova •Palermo La Maddalena •Parma •Pavia Clinica Med.III •Pavia Ematologia •Perugia •Pesaro •Pescara •Piacenza •Pisa •Pordenone •Ragusa •Ravenna •Reggio Calabria •Reggio Emilia •Rionero in Vulture •Roma S.G.Addolorata •Roma Osp. San Giacomo •Roma La Sapienza •Roma Gemelli •Roma Tor Vergata •Roma S.Eugenio •Roma S.Spirito • Roma Campus Biomedico • Roma A.O. Sant’Andrea •Rozzano •S.Felice a Cancello •S.Giovanni Rotondo •Siena •Sondalo •Torino •TorinoM •Udine •Vicenza 78 Centers AGE at Diagnosis SEX PREVIOUS THROMBOSIS Major 4% 2% PREVIOUS HEMORRHAGE 1% Males 40 Females 30 Aims •To registry ET patients observed in the italian Institutions. •To validate the diagnosis appropriateness (WHO criteria) by performing a centralized revision of the bone marrow biopsies •To promote the acquisition of biological data (clonality, JAK2 mutation, haemostasis) •To evaluate the compliance to the therapeutical guide lines of the Italian Society of Haematology •To monitor the ET patients receiving Interferons alpha or Anagrelide •To evaluate cases of pregnancy, pediatric age and familiarity •To create a network for activation of new clinical and biological studies Methods 767 Patients 767 The RIT, co-ordinated by the Haematology Unit of Reggio Emilia, is a web-based registry (www.trombocitemie.it) that, besides a public area, comprehends a database of italian ET patients. The data, with respect of the privacy rules, are object of validation and analysis by various RIT Expert Subcommittees. 20 SYMPTOMS % S. Rupoli, A.Tassetti , N. Cantore, F. Palmieri, V. Liso, G. Specchia, P.Carluccio, C. Cristofalo, C. Gasbarrino, P. Ditonno, E. DE Biasi, E. Cacciola, R. Cacciola, R. Centurioni, A. Inzoli, P. Spedini, A. Bosi, A. M. Vannucchi, E. Antonioli, A. Grossi, F. Balestri, G. Beltrami, R. Bruni, M. Gobbi, I. Pierri, C. Musolino, C. Alati, F. Lussana, S. Caberlon, F. Zallio, P. Mannucci, P. Bucciarelli, A. Zanella, F. Radaelli, A. Iurlo, G. Leonardi, M. Morucci, L. Mastrullo, F. Gonnella, M.R. Villa, D. De Blasi, V. Martinelli, R. Ciancia, G. Gaidano, S. Franceschetti, G. Saglio, D. Cilloni, F. Fabris, M.L. Randi, F. Porretto, M. Crugnola, F. Passamonti, E. Rumi, G. Davì, A. Dragani, E. Orciuolo, G. O. Manenti, G. Garozzo, M. Salvucci, L. Gugliotta, A. Tieghi, C. Musto, L. Maurillo, M. De Muro, M. Cedrone, R. Foà, M. G. Mazzucconi, R. Latagliata, C. Santoro, F. Saltarelli, L. Siracusano, F. Ardizzone, M. Iovine, N. Cascavilla, R. P. Scalzulli, L. Melillo, M. Balboni, A. Candoni, F. Rodeghiero, M. Ruggeri, G. Castaman. PATIENTS 42 % 10 THROMBOTIC RISK FACTORS 69 % 0 0-40 SPLENOMEGALY 25 % PREGNANCIES 60 ASPIRIN 70% True ET (WHO criteria) 60-70 70+ Diagnostic Criteria PLT at Diagnosis CYTOREDUCTION Hydroxyurea Anagrelide Interferons Pipobroman Busulfan 40-60 YEARS 63% 61% 12% 11% 4% 2% <401 100 401-600 601-999 1000-1499 >=1500 80 60 % RIT Panel The Registro Italiano Trombocitemie (RIT) is a web- based registry for the collection of clinical and biological data in ET patients. The RIT is a project approved and supported by the GIMEMA (Gruppo Italiano Malattie Ematologiche dell’Adulto) Characteristic at diagnosis 40 27% 20 Normal KARYOTYPE 274 / 274 0 PVSG Concluding Remarks The majority of the italian Haematological Centers are registering ET patients into the RIT. The preliminary analysis of the 767 registered patients shows that in 92% of patients the diagnosis was done according to the PVSG criteria. In the ET patients, classified according to the WHO criteria, the rate of True ET ( 27%) was similar to that reported in other patient series The ongoing revision of the bone marrow biopsies will permit a reclassification according to the WHO criteria. Improvement of the diagnostic approach is expected and the harvest of biological material as been activated. A separate analysis is ongoing for specific series of patients treated with Anagrelide and Iterferons alpha. WHO OTHER