A patient with idiopathic hypereosinophilic syndrome reacting to a tyrosine-kinase inhibitor

ABSTRACT to participate on ESH International Conference on Myeloproliferative Neoplasms from October 04-06, 2012 Vienna, Austria

Corresponding author:Éva Pósfai, M.D.

2nd Dept. of Int. Med.- University of Szeged, Biological Research Center, Hungarian Academy of Sciences E-mail: evaposfay@gmail.com Telephone: +36 20 4617007

Title : A patient with idiopathic hypereosinophilic syndrome reacting to a tyrosine-kinase inhibitor-

Éva Pósfai¹, Imelda Marton¹, Attila Nemes¹, Anita Kalapos¹, Péter Domsik¹, Zita Borbényi¹

12nd Dept. of Int. Med.- University of Szeged, Hungary

Introduction: The clinical manifestations of HES (hypereosinophilic syndrome) could involve various organs and systems such as heart, skin, lungs, nervous system and gastrointestinal tract. The FIP1L1-PDGFRA fusion gene, the deeper understanding the role of the tyrosine kinase pathway, has changed the standard treatment. Imatinib treatment has dramatically reversed the poor-prognosis. The approximate overall survival has increased from a three-year survival rate of 16% to a five-year survival rate of 80%.

Case presentation- Discussion: Our aim was to present a 33-year old male patient diagnosed 10 years ago, in 2001 with ”HES”. At the time of the diagnosis, the heart and the nervous system were already involved. The patient presented with anaemia, eosinophilia, splenomegaly, PDGFRA positivity, numbness in extremities, right-sided motor function disorder, memory disturbances and dizziness. The myocardium seems to be also affected. The symptoms have been alleviated by steroid therapy but the proportion of eosinophils has not changed in the periphery; thus hydroxyurea has been added. In June 2002, after all investigations were performed, 200 mg imatinib mesylate therapy was initiated considering the results published by Jane Apperley (April 2002). The administration of the therapy resulted rapid regression of the disease, and has led to permanent remission considering the results of our 10-year follow-up.

Conclusions: The new therapy has led to detectable improvement in hematological findings and neurological symptoms and signs. A slower but significant regression in cardiac signs. Looking back at the results of the past 10 years, it can be claimed that imatinib mesylate therapy given in small doses may result in permanent balance in the condition of the patient. However, we have no exact data on how long this therapy should be administered, and whether resistance or relapse may develop toward the drug.