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HOW DIAGNOSTIC AND THERAPEUTIC APPROACHE CHANGED IN THE LAST DECADES IN THE PHILADELPHIA NEGATIVE MPN PATIENTS OF THE REGISTRO ITALIANO TROMBOCITEMIE (RIT)
Author(s): ,
Luigi Gugliotta
Affiliations:
Institute of Hematology 'L. and A. Seragnoli',Policlinico S.Orsola-Malpighi,Bologna,Italy
,
Alessandra Iurlo
Affiliations:
IRCCS Dept. of Hematology,Polyclinic Maggiore Hospital,Milano,Italy
,
Gabriele Gugliotta
Affiliations:
Institute of Hematology 'L. and A. Seragnoli',Policlinico S.Orsola-Malpighi,Bologna,Italy
,
Alessia Tieghi
Affiliations:
IRCCS Dept. of Hematology,Arcispedale Santa Maria Nuova,Reggio Emilia,Italy
,
Anna Candoni
Affiliations:
Udine University,Santa Maria della Misericordia Hospital,Udine,Italy
,
Monia Lunghi
Affiliations:
Piemonte Orientale Univ.UDA Hematology,Medical Sciences 'Amedeo Avogadro',Novara,Italy
,
Alfredo Dragani
Affiliations:
Hematology Dept,Spirito Santo Hospital,Pescara,Italy
,
Elisabetta Antonioli
Affiliations:
Hematology SOD,Univ.Hospital Careggi,Firenze,Italy
,
Potito Rosario Scalzulli
Affiliations:
Hematology Dept,Casa Sollievo della Sofferenza,San Giovanni Rotondo (FG),Italy
,
Vincenzo Martinelli
Affiliations:
Hematology Dept,Univ. Federico II,Napoli,Italy
,
Bruno Martino
Affiliations:
Hematology Dept,AO Bianchi Melacrino Morelli,Reggio Calabria,Italy
,
Maria Luigia Randi
Affiliations:
Internal Medicine,Padova University,Padova,Italy
,
Alessandra Ricco
Affiliations:
Hematology Dept,University, Polyclinic Giovanni XXIII,Bari,Italy
,
Viviana Appolloni
Affiliations:
Hematology Dept,Santa Maria Hospital,Terni,Italy
,
Nilla Maschio
Affiliations:
CMSOE,San Giovanni Apostolo Hospital,Castelfranco Veneto(TV),Italy
,
Raffaele Palmieri
Affiliations:
Hematology Dept,AORN San Giovanni Moscati,Avellino,Italy
,
Maria Langella
Affiliations:
Internal Medicine/OncoHematology Dept.,Umberto I Hospital,Nocera Inferiore,Italy
,
Cristina Santoro
Affiliations:
Hematology Dept,Univ. La Sapienza,Roma,Italy
,
Angela Rago
Affiliations:
Hematology Dept,Univ. Polo Pontino,Latina,Italy
,
Riccardo Ragionieri
Affiliations:
Institute of Hematology 'L. and A. Seragnoli',Policlinico S.Orsola-Malpighi,Bologna,Italy
,
Katia Codeluppi
Affiliations:
IRCCS Dept. of Hematology,Arcispedale Santa Maria Nuova,Reggio Emilia,Italy
,
Emma Cacciola
Affiliations:
Hematology Dept,Ferrarotto Hospital,Catania,Italy
,
Rossella Cacciola
Affiliations:
Hematology Dept,Ferrarotto Hospital,Catania,Italy
,
Lucia Mastrullo
Affiliations:
Hematology Dept,S.Gennaro Hospital,Napoli,Italy
,
Maria Rosaria Esposito
Affiliations:
Hematology Dept,S.Gennaro Hospital,Napoli,Italy
,
Giovanni Caocci
Affiliations:
Hematology Dept,Businco Hospital,Cagliari,Italy
,
Francesco Lanza
Affiliations:
Hematology Dept and CTMO,Istituti Ospedalieri,Cremona,Italy
,
Serena Rupoli
Affiliations:
OncoHematology Dept.,Univ., Ospedali Riuniti Umberto I,Ancona,Italy
,
Ivana Pierri
Affiliations:
Hematology Dept,DIMI,Genova,Italy
,
Ferdinando Porretto
Affiliations:
OncoHematology Dept.,La Maddalena Hospital,Palermo,Italy
,
Angela Melpignano
Affiliations:
Hematology Dept,A. Perrino Hospital,Brindisi,Italy
,
Monica Crugnola
Affiliations:
Onco-Hematology Dept. and CTMO,Maggiore Hospital,Parma,Italy
,
Mauro Di Ianni
Affiliations:
Hematology Dept,Univ. Hospital San Salvatore,L'Aquila,Italy
,
Sara Plebani
Affiliations:
Hematology Dept,University Hospital San Salvatore,L'Aquila,Italy
,
Elisa Molinari
Affiliations:
Hematology Dept,DIMI,Genova,Italy
,
Giuseppina Spinosa
Affiliations:
Hematology Dept.,AO Ospedali Riuniti,Foggia,Italy
,
Marco Santoro
Affiliations:
Hematology Dept. and CTMO,Giaccone Hospital,Palermo,Italy
,
Oreste Villani
Affiliations:
Hematology Dept,CROB,Rionero in Vulture (PZ),Italy
,
Manlio Ricciotti
Affiliations:
Internal Medicine,G. Mazzini Hospital,Teramo,Italy
,
Caterina Musolino
Affiliations:
Hematology Dept.,Messina University,Messina,Italy
,
Emilio Usala
Affiliations:
Hematology Dept.,Businco Hospital,Cagliari,Italy
,
Franca Falzetti
Affiliations:
Hematology Dept.,Perugia University,Perugia,Italy
,
Ercole De Biasi
Affiliations:
Hematology Dept,Civil Hospital,Cittadella (PD),Italy
,
Umberto Santoro
Affiliations:
Statistical Dept.,Bologna University,Bologna,Italy
,
Augusto Federici
Affiliations:
Hematology Dept.,University Hospital,Milano,Italy
,
Nicola Vianelli
Affiliations:
institute of Hematology 'L.A. Seragnoli',Policlinico S. Orsola Malpighi,Bologna,Italy
,
Maria Gabriella Mazzucconi
Affiliations:
Hematology Dept,Univ. La Sapienza,Roma,Italy
Giorgina Specchia
Affiliations:
Hematology Dept,Universitary Polyclinic Giovanni XXIII,Bari,Italy
(Abstract release date: 05/21/15) EHA Library. Gugliotta L. 06/12/15; 102910; PB1919 Disclosure(s): Policlinico S.Orsola-Malpighi
Institute of Hematology 'L. and A. Seragnoli'
Dr. Luigi Gugliotta
Dr. Luigi Gugliotta
Contributions
Abstract
Abstract: PB1919

Type: Publication Only

Background
The last decade starts for the patients with Philadelphia Neg- MPN after the JAK2 mutations discovery (2005), the Italian guidelines for Essential Thrombocythemia therapy (2004), and the WHO classification (2001) 

Aims
To evaluate how the diagnostic and therapeutic  approach changed in the clinical practice in the last decade.

Methods
The patients of the Registro Italiano Trombocitemie (RIT) diagnosed before and after 2005 were compared by considering demography, clinico-biological characteristics at diagnosis, and the initial treatment approach.

Results
The 2418 analyzed patients were diagnosed (PVSG or WHO criteria) before 2005 (Group I, n 1230, 51%) and after 2005 (Group II, n 1188, 49%). The patients of Group II, as compared with those of Group I, had at diagnosis older age (median 60 vs 55 y, p60 y in 50% vs 41%, p<0.001), higher rate of comorbidities (56% vs 49%, p 0.002), and lower median PLT count (748 vs 786 x109/L, p< 0.001). No significant differences were found for gender (males 38% vs 38%), prior thrombosis (20% vs 20%), prior hemorrhage (4.9% vs 5.6%), CV general risk factors (71% vs 68%), symptoms (41% vs 41%), splenomegaly (25% vs 23%), hepatomegaly (22% vs 24%), WBC count (median 8.7 vs 8.5 x109/L; >10 x109/L 28% vs 27%), and HCT level (median in males 44.8% vs 44.1%; median in females 41.5% vs 41.0%). The bone marrow biopsy in the first year was performed more frequently (76% vs 67%, p<0.001). The test for JAK2 mutation was performed in 89% of Group II patients, and, a posteriori, in 72% of Group I patients (p <0.001). The patients of Group II had a higher rate of JAK2 V617F mutation (62% vs 56%, p 0.015), and of allele burden <25% (65% vs 51%, p <0.01). Moreover, the patients of Group II had a higher rate of standard high thrombotic risk (57% vs 49%, p<0.001).During the first year after diagnosis, an antiplatelet (+ or - cytoreductive) treatment (almost always low dose aspirin) was started more frequently in the Group II (65% vs 48%, p<0.001), particularly (p<0.001) in patients at low thrombotic risk (standard and IPSET-Th). Similarly, a cytoreductive (+ or - antiplatelet) treatment was started more frequently in the Group II (57% vs 50%, p 0.002), particularly (74% vs 68%, p 0.013) in patients with high thrombotic risk (standard and IPSET-Th). In the Group II, as compared with Group I, the rate of hydroxycarbamide use was higher for patients over 60 y (71% vs 63%, p<0.01) and lower for those below 40 y (8% vs 14%, p<0.05), while the rate of anagrelide use was higher for patients below 40 y (6.4% vs 2.7%, p<0.05).

Summary
The patients of the RIT diagnosed after 2005, compared with those diagnosed before, although were older, had more frequently a bone marrow biopsy at diagnosis. They had lower platelet count, higher rate of JAK2 V617F mutation, and higher rate of high thrombotic risk. No significant differences were found for gender, prior thrombosis, CV general risk factors, symptoms, splenomegaly, WBC count. During the 1st year, they were treated more frequently with antiplatelet drugs (particularly if at low thrombotic risk), and with cytoreductive drugs (particularly if at high thrombotic risk). The hydroxycarbamide use increased in older patients, while the anagrelide use increased in younger ones. In conclusion, in the real life of the RIT centers, a significant improvement of the diagnostic and therapeutic approach was documented in the last decade.

Keyword(s): Diagnosis, Platelet, Therapy, Thrombocythemia

Session topic: Publication Only
Abstract: PB1919

Type: Publication Only

Background
The last decade starts for the patients with Philadelphia Neg- MPN after the JAK2 mutations discovery (2005), the Italian guidelines for Essential Thrombocythemia therapy (2004), and the WHO classification (2001) 

Aims
To evaluate how the diagnostic and therapeutic  approach changed in the clinical practice in the last decade.

Methods
The patients of the Registro Italiano Trombocitemie (RIT) diagnosed before and after 2005 were compared by considering demography, clinico-biological characteristics at diagnosis, and the initial treatment approach.

Results
The 2418 analyzed patients were diagnosed (PVSG or WHO criteria) before 2005 (Group I, n 1230, 51%) and after 2005 (Group II, n 1188, 49%). The patients of Group II, as compared with those of Group I, had at diagnosis older age (median 60 vs 55 y, p60 y in 50% vs 41%, p<0.001), higher rate of comorbidities (56% vs 49%, p 0.002), and lower median PLT count (748 vs 786 x109/L, p< 0.001). No significant differences were found for gender (males 38% vs 38%), prior thrombosis (20% vs 20%), prior hemorrhage (4.9% vs 5.6%), CV general risk factors (71% vs 68%), symptoms (41% vs 41%), splenomegaly (25% vs 23%), hepatomegaly (22% vs 24%), WBC count (median 8.7 vs 8.5 x109/L; >10 x109/L 28% vs 27%), and HCT level (median in males 44.8% vs 44.1%; median in females 41.5% vs 41.0%). The bone marrow biopsy in the first year was performed more frequently (76% vs 67%, p<0.001). The test for JAK2 mutation was performed in 89% of Group II patients, and, a posteriori, in 72% of Group I patients (p <0.001). The patients of Group II had a higher rate of JAK2 V617F mutation (62% vs 56%, p 0.015), and of allele burden <25% (65% vs 51%, p <0.01). Moreover, the patients of Group II had a higher rate of standard high thrombotic risk (57% vs 49%, p<0.001).During the first year after diagnosis, an antiplatelet (+ or - cytoreductive) treatment (almost always low dose aspirin) was started more frequently in the Group II (65% vs 48%, p<0.001), particularly (p<0.001) in patients at low thrombotic risk (standard and IPSET-Th). Similarly, a cytoreductive (+ or - antiplatelet) treatment was started more frequently in the Group II (57% vs 50%, p 0.002), particularly (74% vs 68%, p 0.013) in patients with high thrombotic risk (standard and IPSET-Th). In the Group II, as compared with Group I, the rate of hydroxycarbamide use was higher for patients over 60 y (71% vs 63%, p<0.01) and lower for those below 40 y (8% vs 14%, p<0.05), while the rate of anagrelide use was higher for patients below 40 y (6.4% vs 2.7%, p<0.05).

Summary
The patients of the RIT diagnosed after 2005, compared with those diagnosed before, although were older, had more frequently a bone marrow biopsy at diagnosis. They had lower platelet count, higher rate of JAK2 V617F mutation, and higher rate of high thrombotic risk. No significant differences were found for gender, prior thrombosis, CV general risk factors, symptoms, splenomegaly, WBC count. During the 1st year, they were treated more frequently with antiplatelet drugs (particularly if at low thrombotic risk), and with cytoreductive drugs (particularly if at high thrombotic risk). The hydroxycarbamide use increased in older patients, while the anagrelide use increased in younger ones. In conclusion, in the real life of the RIT centers, a significant improvement of the diagnostic and therapeutic approach was documented in the last decade.

Keyword(s): Diagnosis, Platelet, Therapy, Thrombocythemia

Session topic: Publication Only

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