THE NEW ERA OF THALASSEMIA SYNDROMES: IS IT TIME TO

Transcript

THE NEW ERA OF THALASSEMIA SYNDROMES: IS IT TIME TO
Invited Speakers:
Kunle Adekile
M. B. Agarwal
Abdulla Al Jefri
Yesim Aydinok
Caterina Borgna Pignatti
Maria Domenica Cappellini
Marina Cavazzana-Calvo
Adriana Ceci
Alan Cohen
Shahina Daar
Vito Di Marco
Amal El-Beshlawy
John Fang
Aldo Filosa
Gian Luca Forni
Suthat Fucharoen
Mahmoud Hajipour
Olivier Hermine
Ibrahim Hishamshah
Mehran Karimi
Antonis Kattamis
Aurelio Maggio
Khaled Musallam
Dudley Pennell
Alessia Pepe
Antonio Piga
John Porter
Vijay G. Sankaran
Farrukh Shah
Alok Srivastava
Ali Taher
Paul Telfer
Vip Viprakasit
... Who
John Wood
Mahmoud Yassin
Kuwait
India
KSA
Turkey
Italy
Italy
France
Italy
USA
Oman
Italy
Egypt
China
Italy
Italy
Thailand
Iran
France
Malaysia
Iran
Greece
Italy
Uk UAE
Uk
Italy
Italy
Uk
USA
KSA
India
Lebanon
Uk
Thailand
...
...
Qatar
CME Provider and Organizing Secretariat
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THE NEW ERA OF
THALASSEMIA SYNDROMES:
IS IT TIME TO REVISIT CLINICAL
CLASSIFICATION OF THE THALASSEMIAS
?
September 15-16, 2017
CAMPUS OF HEMATOLOGY "Franco e Piera Cutino"
A.O.R. "Villa Sofia - V. Cervello"
Palermo (Italy)
FIRST
ANNOUNCEMENT
Chairmen:
Antonis Kattamis, Ali Taher, Khaled Musallam and Aurelio Maggio
Rationale
Recent data suggest that the survival of well-treated patients with Thalassemia
Major is now similar to that of Intermediate Thalassemia Intermedia (Vitrano et
al., 2016, BJH). In addition, retrospective data on 4.943 subjects with a heterozygote state of beta-thalassemia, suggest that, even in patients defined as thalassemia
carriers (Thalassemia Minor), there is an increase in morbidity (cirrhosis, kidney
disease, etc.) compared with the non-thalassemic population of subjects not
heterozygote (Graffeo et al., AJH, in press). These data call for a potential revision
of the clinical classification of thalassemia based on strict categories of severity
towards a classification including a "continuum" of the same disease divided into
that manifests in stages. The advantages of this new classification would consist of:
1) expand the use of chelation therapy and transfusion to the ‘traditionally’ less
severe forms of thalassemia syndromes or whose management was not commonly
considered; 2) reduce the morbidity and mortality of such less severe forms of
thalassemia syndromes; 3) reduce health care costs associated with the treatment of
these uncontrolled complications (cirrhosis, etc.) through prevention; 4) expand
the number of patients who are eligible to innovative therapies; 5) review the
indications for prenatal diagnosis in Thalassemia Major.
Main Topics
• Thalassemia Major: how had conventional treatment been changing survival
• Current survival of thalassemia in eastern countries: the Iran experience
• Thalassemia Intermedia: is disease morbidity observed today less severe
compared with thalasssemia major ?
• The role of current chelation treatment in improving prognosis of thalassemia major and intermedia
• The impact of new direct anti-viral treatment in improving prognosis of
thalassemia syndromes
• What is the impact of novel chelators or new formulations of available
chelators treatments on outcomes in thalassemia syndromes?
• Current state of total body iron burden in thalassemia major and thalassemia intermedia determined by MRI: what is the difference in terms of liver
iron concentration?
• Heart MRI T2* signal: a crucial tool for improving prognosis in thalassemia
• Transfusion therapy: what has changed with respect to safety and control of
iron loading?
• How much are novel innovative therapies expected to influence the prognosis of thalassemia syndromes ?
• Gene therapy for thalassemia and sickle-cell-disease: what is the future
impact of this procedure on these diseases ?
• “Cluster analysis” to differentiate thalassemia syndromes: report on 7843
subjects.
Round Table:
this will have the participation of all speakers to discuss and reconsider the
current classification of thalassemia.
Main Topics:
Is the current dichotomy classification as thalassemia major and intermedia appropriate
for management of these diseases?
Is current access to thalassemia care limited by this current dichotomy classification?
Is current availability of chelators a barrier for application of transfusion regimens in less
severe phenotypes of thalassemia?
Would re-classification of thalassemia in stages improve or worsen management
outcomes?
Could such new classification be more useful for allowing wider use of innovative
treatment for thalassemia?
The results of this meeting will be published as Special Issue on
Anemia Journal!