title: Myelofibrosis successfully treated with prednisolone in a patient with pachydermoperiostosis.
abstract: Pachydermoperiostosis (PDP) is a rare disorder of bone and connective tissue growth. A 21-year-old man was referred to our hospital with anemia. He showed characteristics of PDP. Bone marrow biopsy showed myelofibrosis. Chromosomal abnormalities or JAK2 mutation were not found. Anemia gradually progressed, and he became transfusion-dependent. Oral prednisolone was initiated; it gradually improved his anemia and rendered the patient free of transfusion. However, other clinical symptoms such as clubbed fingers and skin hypertrophy remained unimproved. In this case, the serum concentration of vascular endothelial growth factor and transforming growth factor-β levels were increased. Further investigation will be necessary to establish appropriate treatment strategies for this disease.
authors.affiliation: The First Department of Internal Medicine, Gifu University Graduate School of Medicine, Japan.
authors.forename: Soranobu
authors.initials: S
authors.lastname: Ninomiya
authors.forename: Takeshi
authors.initials: T
authors.lastname: Hara
authors.forename: Hisashi
authors.initials: H
authors.lastname: Tsurumi
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Kanemura
authors.forename: Senji
authors.initials: S
authors.lastname: Kasahara
authors.forename: Yoko
authors.initials: Y
authors.lastname: Ogawa
authors.forename: Mariko
authors.initials: M
authors.lastname: Seishima
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Hirose
authors.forename: Hisataka
authors.initials: H
authors.lastname: Moriwaki
mesh_terms: D000740:Anemia / Q000150:complications / Q000188:drug therapy; D001853:Bone Marrow / Q000473:pathology; D004252:DNA Mutational Analysis; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D010004:Osteoarthropathy, Primary Hypertrophic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D010005:Osteoarthropathy, Secondary Hypertrophic / Q000150:complications; D011239:Prednisolone / Q000627:therapeutic use*; D055728:Primary Myelofibrosis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D016212:Transforming Growth Factor beta / Q000097:blood; D042461:Vascular Endothelial Growth Factor A / Q000097:blood; D055815:Young Adult
chemical_list: D016212:Transforming Growth Factor beta; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D011239:Prednisolone; C507924:JAK2 protein, human; D053614:Janus Kinase 2