title: Liver fibrosis with hypereosinophilia causing transient abnormal myelopoiesis.
abstract: Transient abnormal myelopoesis is mostly self-resolving and has a good prognosis, but some patients subsequently die of liver fibrosis. We report the case of an infant with Down syndrome who developed life-threatening liver fibrosis at the same time as the blasts were about to disappear. This patient also had a marked increase in eosinophils, which were possibly harboring a GATA1 mutation and were expressing a high level of platelet-derived growth factor-B mRNA; these may have been involved in the development of liver fibrosis. Low-dose cytosine arabinoside therapy effectively treated both hypereosinophilia and liver fibrosis.
authors.affiliation: Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Minakata
authors.affiliation: Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Sakata
authors.affiliation: Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
authors.forename: Norihisa
authors.initials: N
authors.lastname: Wada
authors.affiliation: Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
authors.forename: Yuhei
authors.initials: Y
authors.lastname: Konishi
authors.affiliation: Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Marutani
authors.affiliation: Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
authors.forename: Takuji
authors.initials: T
authors.lastname: Enya
authors.affiliation: Department of Pediatrics, Yodogawa Christian Hospital, Osaka City, Osaka, Japan.
authors.forename: Hidenori
authors.initials: H
authors.lastname: Nakagawa
authors.affiliation: Department of Pediatrics, Yodogawa Christian Hospital, Osaka City, Osaka, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Wada
authors.affiliation: Department of Pediatrics, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Takemura
mesh_terms: D001706:Biopsy; D004247:DNA / Q000235:genetics; D004252:DNA Mutational Analysis; D004314:Down Syndrome / Q000150:complications / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics; D004802:Eosinophilia / Q000150:complications* / Q000175:diagnosis; D050982:GATA1 Transcription Factor / Q000235:genetics / Q000378:metabolism; D006801:Humans; D007231:Infant, Newborn; D007955:Leukemoid Reaction / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics; D008103:Liver Cirrhosis / Q000150:complications* / Q000175:diagnosis; D008297:Male; D038042:Myelopoiesis
chemical_list: D050982:GATA1 Transcription Factor; C494688:GATA1 protein, human; D004247:DNA
keywords: eosinophilia; liver fibrosis; low-dose cytosine arabinoside; transient abnormal myelopoiesis