Myelofibrosis in children: experience at a single tertiary care center in India.
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.
Arya
Laxman Singh
LS
Division of Pediatric Hematology-Oncology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India. lsarya@rediffmail.com
Thavraj
Vasantha
V
Chandra
D
D
Kulkarni
Ketan Prasad
KP
Kumar
R
R
Dawar
R
R
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