MYELOFIBROSIS IN CHILDREN: Experience at a Single Tertiary Care Center in India

MYELOFIBROSIS IN CHILDREN: Experience at a Single Tertiary Care Center in India

Laxman Singh Arya,Vasantha Thavraj,D. Chandra,Ketan Prasad Kulkarni,R. Kumar,R. Dawar;Laxman Singh Arya;Vasantha Thavraj;D. Chandra;Ketan Prasad Kulkarni;R. Kumar;R. Dawar;
pediatric hematology and oncology 2010 Vol. 27 pp. 355-362
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dawar2010pediatricmyelofibrosis

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.

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