title: Molecular analysis of chronic eosinophilic leukemia with t(4;10) showing good response to imatinib mesylate.
abstract: A 38-year-old Japanese man was referred to our hospital in June 2003 for treatment of acute respiratory failure with severe eosinophilia. Idiopathic hypereosinophilic syndrome had been diagnosed in 1994. However, karyotypic examination of bone marrow cells revealed that chromosomal translocation with t(4;10)(q12;p11) had occurred in 2000, and chronic eosinophilic leukemia was diagnosed. At admission, the patient's respiratory condition was extremely serious, and mechanical support was necessary. Despite treatment with steroid pulse therapy and cytarabine, the blood eosinophil count did not decrease, and the patient's respiratory condition worsened. After obtaining informed consent, we administered imatinib mesylate at a dose of 200 mg/day for 2 days and 100 mg/day for 3 days. The blood eosinophil count decreased dramatically over 5 days, and the patient's condition rapidly improved, such that the patient could be discharged. In this case, we performed molecular analysis using peripheral blood. The FIP1-like 1 (FIP1L1)-platelet-derived growth factor receptor alpha (PDGFRalpha) fusion transcript was found with the reverse transcriptase polymerase chain reaction analysis. In this case, eosinophilia was possibly caused by constitutive activation of tyrosine kinase produced by the FIP1L1-PDGFRalpha fusion transcript.
authors.affiliation: Department of Hematology/Oncology, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Haruko
authors.initials: H
authors.lastname: Tashiro
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Shirasaki
authors.forename: Mitsuho
authors.initials: M
authors.lastname: Noguchi
authors.forename: Moritaka
authors.initials: M
authors.lastname: Gotoh
authors.forename: Kazuo
authors.initials: K
authors.lastname: Kawasugi
authors.forename: Naoki
authors.initials: N
authors.lastname: Shirafuji
mesh_terms: D000328:Adult; D044466:Asian People; D001549:Benzamides; D002879:Chromosomes, Human, Pair 10 / Q000235:genetics; D002894:Chromosomes, Human, Pair 4 / Q000235:genetics; D002908:Chronic Disease; D004789:Enzyme Activation / Q000187:drug effects; D004802:Eosinophilia / Q000188:drug therapy / Q000201:enzymology / Q000235:genetics; D005500:Follow-Up Studies; D015971:Gene Expression Regulation, Enzymologic / Q000187:drug effects; D015973:Gene Expression Regulation, Leukemic / Q000187:drug effects; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy* / Q000201:enzymology* / Q000235:genetics; D000068877:Imatinib Mesylate; D007958:Leukocyte Count; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000037:antagonists & inhibitors* / Q000096:biosynthesis; D010879:Piperazines / Q000008:administration & dosage*; D047428:Protein Kinase Inhibitors / Q000008:administration & dosage*; D011743:Pyrimidines / Q000008:administration & dosage*; D012333:RNA, Messenger / Q000096:biosynthesis / Q000235:genetics; D012334:RNA, Neoplasm / Q000096:biosynthesis / Q000235:genetics; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000037:antagonists & inhibitors* / Q000096:biosynthesis; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods; D013997:Time Factors; D014178:Translocation, Genetic / Q000235:genetics; D039221:mRNA Cleavage and Polyadenylation Factors / Q000037:antagonists & inhibitors* / Q000096:biosynthesis
chemical_list: D001549:Benzamides; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D012333:RNA, Messenger; D012334:RNA, Neoplasm; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha