Practice guidelines for the therapy of essential thrombocythemia. A statement from the Italian Society of Hematology, the Italian Society of Experimental Hematology and the Italian Group for Bone Marrow Transplantation.
OBJECTIVE
The Italian Society of Hematology (SIE) and the two affiliated Societies (SIES and GITMO) commissioned a project to develop guidelines for the therapy of essential thrombocythemia (ET) using evidence-based knowledge and consensus formation techniques.


METHODS
Key questions on the optimal management of ET patients were formulated by an Advisory Council (AC) and approved by an Expert Panel (EP) composed of 7 senior hematologists. The AC systematically reviewed the published literature from 1980 to August 2002, and articles were graded according to their internal validity and quality. Using the Delphi technique, the EP was asked to answer the key questions according to the available evidence. From September 2002 to March 2003, four Consensus Conferences were held in accordance with the Nominal Group Technique with the goal of solving residual disagreement on recommendations.


RESULTS
The EP provided recommendations on when to start platelet-lowering therapy, the most appropriate platelet-lowering agent, the use of anti-platelet therapy, and the management of women in childbearing age and of pregnant women.


CONCLUSIONS
By using evidence and consensus, recommendations for the treatment of key problems in ET have been issued. Statements are graded according to the strength of the supporting evidence and uncertainty is explicitly declared.
Divisione di Ematologia. Spedali Riuniti, Bergamo, Italy.
Tiziano
T
Barbui
Giovanni
G
Barosi
Alberto
A
Grossi
Luigi
L
Gugliotta
Lucio N
LN
Liberato
Monia
M
Marchetti
Maria Gabriella
MG
Mazzucconi
Francesco
F
Rodeghiero
Sante
S
Tura
D000208:Acute Disease; D000328:Adult; D000368:Aged; D000477:Alkylating Agents / Q000009:adverse effects / Q000627:therapeutic use; D002471:Cell Transformation, Neoplastic; D002648:Child; D002986:Clinical Trials as Topic; D015331:Cohort Studies; D018450:Disease Progression; D019317:Evidence-Based Medicine; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006470:Hemorrhage / Q000517:prevention & control; D006801:Humans; D006918:Hydroxyurea / Q000009:adverse effects / Q000627:therapeutic use; D007231:Infant, Newborn; D007951:Leukemia, Myeloid / Q000209:etiology / Q000517:prevention & control; D008297:Male; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000009:adverse effects / Q000627:therapeutic use; D011247:Pregnancy; D011256:Pregnancy Outcome; D016019:Survival Analysis; D013920:Thrombocythemia, Essential / Q000188:drug therapy* / Q000628:therapy; D019851:Thrombophilia / Q000150:complications / Q000235:genetics; D013927:Thrombosis / Q000517:prevention & control; D016896:Treatment Outcome
D000477:Alkylating Agents; D005343:Fibrinolytic Agents; D010975:Platelet Aggregation Inhibitors; D006918:Hydroxyurea