title: Myelofibrosis in children: experience at a single tertiary care center in India.
abstract: The authors describe 10 cases of myelofibrosis diagnosed and managed at their center over 16 years. There were 2 and 8 cases, respectively, of primary and secondary myelofibrosis. All patients presented with fever, pallor, hepatosplenomegaly, and/or lymphadenopathy. Hodgkin's lymphoma (n = 4), neuroblastoma (n = 1), thrombasthenic thrombopathy (n = 1), and retroperitoneal-mass (n = 1) were causal in 7 patients, whereas the diagnosis could not be established in a sole case of secondary myelofibrosis. Patients were managed with chemotherapy and appropriate care. However, outcome was poor. The authors emphasize variable clinical-laboratory spectrum of myelofibrosis, highlight management concerns, and demonstrate that prognosis/outcome depends upon appropriate management of the underlying condition.
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India. lsarya@rediffmail.com
authors.forename: Laxman Singh
authors.initials: LS
authors.lastname: Arya
authors.forename: Vasantha
authors.initials: V
authors.lastname: Thavraj
authors.forename: D
authors.initials: D
authors.lastname: Chandra
authors.forename: Ketan Prasad
authors.initials: KP
authors.lastname: Kulkarni
authors.forename: R
authors.initials: R
authors.lastname: Kumar
authors.forename: R
authors.initials: R
authors.lastname: Dawar
mesh_terms: D002648:Child; D019468:Disease Management; D004358:Drug Therapy; D006689:Hodgkin Disease / Q000150:complications; D006801:Humans; D007194:India; D009447:Neuroblastoma / Q000150:complications; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D012186:Retroperitoneal Neoplasms / Q000150:complications; D013915:Thrombasthenia / Q000150:complications; D016896:Treatment Outcome