Myelofibrosis in children: experience at a single tertiary care center in India

Pediatr Hematol Oncol. 2010 Aug;27(5):355-62. doi: 10.3109/08880011003739430.

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.

MeSH terms

  • Child
  • Disease Management
  • Drug Therapy
  • Hodgkin Disease / complications
  • Humans
  • India
  • Neuroblastoma / complications
  • Primary Myelofibrosis / diagnosis
  • Primary Myelofibrosis / drug therapy*
  • Primary Myelofibrosis / etiology*
  • Retroperitoneal Neoplasms / complications
  • Thrombasthenia / complications
  • Treatment Outcome