ISTH 2015

264-MON-PO Faenza.; 8 Piacenza. Piacenza. 8 Faenza.; - Ravenna 7 Piacenza; 6 Ferrara; 5 Emilia; Reggio 4 Cesena; 3 Bologna; 2 Parma; 1

* RER HEMOPHILIA CENTERS HEMOPHILIA RER *

assistance monitoring and analyzing the quality of life and treatment costs. treatment and life of quality the analyzing and monitoring assistance of quality reporting, epidemiological detailed of means by care of quality improved Registry RER and network HC opinion, our In

s treatment. Moreover, more concentrate consumption were recorded with a considerable reduction in bleeds. in reduction considerable a with recorded were consumption concentrate more Moreover, treatment. s axi prophyl of use and diagnoses genetic detection, inhibitor (138%), patients enrolled of increase an was there 2003 to Compared

CONCLUSION

AnnaritaTagliaferri Hemophilia- clinical

Fig. 5 Fig.

Tab. 2 Tab.

324 12.000 2.595 96.000 37 429 3.000 0 0 7 HB Mild

19.235 654.000 18.176 618.000 34 2.542 61.000 1.792 43.000 24 HB Moderate

Fig. 4 Fig.

9.095 191.000 108.857 2.286.000 21 5.024 85.400 68.412 1.163.000 17 HB Severe

1.718 268.000 4.035 629.500 156 194 18.000 274 25.500 93 HA Mild

15.525 91.6000 60.585 3.574.500 59 1.370 63.000 16.609 764.000 46 HA Moderate

27.083 3.250.000 201.231 24.147.750 120 8.741 708.000 63.840 5.171.000 81 HA Severe

UI/pts/year UI UI/pts/year UI UI/pts/year UI UI/pts/year UI

of patients of ° N patients of ° N

Plasmaderived Recombinant Plasmaderived Recombinant

2014 2003 Diagnosis

Tab 1 Tab Fig. 2 b 2 Fig.

473 517 595 67 107 313 Total

22 26 32 2 2 3 HB Carrier

118 122 136 6 6 30 HA Carrier

17 19 37 2 2 7 HB Mild

28 30 34 11 11 24 HB Moderate

19 20 21 7 8 17 HB Severe

110 132 156 0 4 93 HA Mild

43 51 59 3 19 46 HA Moderate

112 113 120 36 55 81 HA Severe

performed performed

characterization characterization

of patients of ° N of patients of ° N

Molecular Molecular Molecular Molecular Genetic Test Genetic Genetic Test Genetic

2003 Diagnosis 2014

. patients the of number the of rise and Immunetolerance

record, clinical in patients by done treatment, home the of recording better a prophylaxis, of spread

Fig 2 a 2 Fig

DOI:10.3252/pso.eu.ISTH2015.2015 3 Fig. the to due was patient/year per IU 234 , 119 to 728 , 72 from ) 2 . (Tab consumption concentrate of increase The

2014 2013 2012 2011 2010 2009 2008 2007 2006 2005 2004 2003

. ) 5 . (Fig % 50 by decreased bleeds the

0

while ), 4 . (Fig % 4 . 51 to % 7 . 27 from Hemophilia severe with patients among spread regimen Prophylaxis

34

36

23

41

30

32 32 36 35

43

38 58 51

63

65

32

76

49

70

73

40 71 112 42

83

46 100

67 114

116

200

90 . ) 3 . (Fig HB 1 and HA 27 were 2014 in while HA, 11 were patients inhibitor 2003 In

114

124

147

155 163

. ) 1 (Tab Parma of Centre HUB the of laboratory genetic the to thanks

400

372

, % 86 to % 34 from increased patients HB and HA moderate and severe of characterization molecular The 415

442

467

501

. b) 2 . (Fig HC at enrolment the in and diagnosis 532 600

495

587

553

653 the in accuracy better a showing ) % 18 to 10 ( 207 to 51 from with Disorders Bleeding Rare mostly but

587

621 712

800

667

, 375 to 142 from VWD , 427 to 268 from HB) (HA, B and A Hemophilia : rose diagnosis all of number The

726

761

784

798

852 . times) 08 . 1 ( 000 , 446 , 4 to 000 , 098 , 4 from increased inhabitants RER of number the while

1000 872

945

a) 2 . (Fig times) 3 . 2 ( 1177 to 495 from increased HC the at up followed patients IBD the 2014 to 2003 From

> 6 5 y 5 6 > y 64 - 18 y 17 - 7 y 6 0-

presented Poster 1038 1200

RESULTS 1103 1177

(Fig 1). Great efforts are made to ensure high quality of data collection. collection. data of quality high ensure to made are efforts Great 1). (Fig months six every ) www.registroemofiliarer.it ( Fig. 1 Fig.

(HC), organized in a ‘Hub and Spoke’ network, to share database, to extract detailed data and publish them on the registry registry the on them publish and data detailed extract to database, share to network, Spoke’ and ‘Hub a in organized (HC), Centres Haemophilia 8 the allow to created was ®) xl’Emofilia ( record clinical based - at web A

: METHODS

. trend cost health and aspects epidemiologic

social, and life of quality related health complications, its and disease the tracking patients manage to how understand better to is register the of aim primary The . system this of results year 12 the provide We

AIMES

Disorders (IBD). Disorders

In 2003 the Health Authority of Emilia of Authority Health the 2003 In Romagna Region (RER), developed a web a developed (RER), Region Romagna - based registry to monitor epidemiology, treatment c treatment epidemiology, monitor to registry based - osts and improve the quality of health care of patients with Inherited Bleeding Bleeding Inherited with patients of care health of quality the improve and osts

BACKGROUND

Network of Haemophilia Centres Centres Haemophilia of Network Emilia Romagna Region*. Italy Region*. Romagna Emilia -

. M . A Pizzini , . C Biasoli , . L Valdrè , . G Rodorigo , . G Quintavalle , . F Riccardi , . C Perna Di , . F . G Rivolta . A Tagliaferri , . P Pedrazzi , . S Macchi , . C . M Arbasi , . L . M Serino ,

3 2 2 1 1 1 1 1 8 7 6 5 4

OF THE EMILIA ROMAGNA REGION IN ITALY IN REGION ROMAGNA EMILIA THE OF

TWELVE YEAR RESULTS OF INHERITED BLEEDING DISORDERS REGISTRY REGISTRY DISORDERS BLEEDING INHERITED OF RESULTS YEAR TWELVE

2003 2014 -

s r e d r o s i D g n i d e e l B d e t i r e h n I

r o f

e r t n e C e c n e r e f e R l a n o i g e R