title: Idiopathic myelofibrosis with generalized periostitis in a 4-year-old girl.
abstract: Idiopathic myelofibrosis, a chronic myeloproliferative disorder of unknown origin, is characterized by splenomegaly, extramedullary hematopoiesis, leukoerythroblastosis, teardrop erythrocytes, and myelofibrosis. It is a rare disorder in childhood. The authors describe a 4-year-old girl with features consistent with idiopathic myelofibrosis, who also had generalized solid laminated periosteal reaction involving all long bones. The presence of thrombocytopenia at the onset and lack of leukocytosis were in contrast to the reported features seen in children. Recent case reports describe a relatively indolent course in children. Spontaneous remissions have also been described in pediatric cases. The fulminant course of this patient without any features of malignant transformation was noteworthy in this regard.
authors.affiliation: Department of Pediatrics, V. M. Medical College & Safdarjang Hospital, New Delhi, India. mandeepkaurwalia@hotmail.com
authors.forename: Mandeep
authors.initials: M
authors.lastname: Walia
authors.forename: Rajesh
authors.initials: R
authors.lastname: Mehta
authors.forename: Premila
authors.initials: P
authors.lastname: Paul
authors.forename: Sumita
authors.initials: S
authors.lastname: Saluja
authors.forename: Sujala
authors.initials: S
authors.lastname: Kapoor
authors.forename: Monika
authors.initials: M
authors.lastname: Sharma
mesh_terms: D001853:Bone Marrow / Q000473:pathology; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007194:India; D010522:Periostitis / Q000175:diagnosis* / Q000473:pathology; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000473:pathology