The purpose of this study is to assess the clinical anti-proliferative activity of STI571 (Glivec®, Novartis, Pharma) in patients with HES defined as: 1. Idiopathic Hypereosinophilic Syndrome (secondary HES), defined as a peripheral blood eosinophilia greater than 1,500 cells/µL for longer than 6 months, absence of other apparent aetiologies for eosinophilia and with or without signs and symptoms of organ involvement, irrespective to expression of any of imatinib targets (c-Kit receptor, PDGFR, bcr-abl receptor) on bone marrow cells. 2. Familiar hypereosinophilia defined as a peripheral blood eosinophilia greater than 1,500 cells/µL for longer than 6 months, absence of other apparent aetiologies for eosinophilia and signs and symptoms of organ involvement, irrespective to expression of any of imatinib targets (c-Kit receptor, PDGFR, bcr-abl receptor) on bone marrow cells, and with a recognized or reported cases of hypereosinophilia in the patient's family. 3. Chronic myeloproliferative disorder, defined as chronic eosinophilic leukemia (CEL) with the presence of blasts (\>10%) in the bone marrow (BM), or the presence of immature eosinophils in different tissues, or an aggressive clinical course or the presence of clonal cytogenetic anomalies. 4. Myeloproliferative disorder (MPD) with eosinophilia, eosinophilic leukemia or chronic myelomonocytic leukemia \[myeloproliferative disorders/myelodysplastic syndromes (MPD/MDS)\] with evidence of: * t(5;12)(q33;p13) by cytogenetic or fluorescent in situ hybridization (FISH) analysis, or * ETV6/TEL-PDGFRB fusion transcript by reverse transcription polymerase chain reaction (RT-PCR), or * PDGFRB disruption, assessed or suspected, by other translocations with additional partner genes (H4, HIP1, CEV14 and Rab5) 5, or * MPD/MDS who have constitutive activation of the gene for platelet-derived growth factor receptor beta (PDGFRB) 6 by point mutations
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Masking
NONE
Istituto di Ematologia e Oncologia Medica "L. e A. Seràgnoli" Università degli Studi di Bologna
Bologna, Italy
RECRUITINGDipartimento di Medicina Interna - Università di Genova
Genova, Italy
RECRUITINGDipartimento di Biochimica e Biotecnologie Mediche - Università degli Studi di Napoli "Federico II"
Napoli, Italy
RECRUITINGDivisione di Ematologia - Università degli Studi di Napoli "Federico II"
Napoli, Italy
RECRUITINGS.C. Medicina Interna II ed Ematologia - Laboratorio di Medicina Interna e Molecolare - A.O. San Luigi
Orbassano, Italy
RECRUITINGDivisione di Ematologia - IRCCS Policlinico S. Matteo
Pavia, Italy
RECRUITINGU.O. Ematologia - Dipartimento di Oncologia ed Ematologia, Presidio Ospedaliero di Ravenna
Ravenna, Italy
RECRUITINGU.O. Ematologia - Arcispedale Santa Maria Nuova
Reggio Emilia, Italy
RECRUITINGCattedra di Ematologia - Università "Tor Vergata"
Roma, Italy
RECRUITINGCattedra di Ematologia - Università "La Sapienza"
Roma, Italy
RECRUITING...and 4 more locations
Rate of complete response (CR) in all patients at 3rd month
Duration of response (CR)
Overall survival at 12th month
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