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),...

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Published in:Pediatric hematology and oncology 2010-07, Vol.27 (5), p.355-362
Main Authors: Arya, Laxman Singh, Thavraj, Vasantha, Chandra, D., Kulkarni, Ketan Prasad, Kumar, R., Dawar, R.
Format: Article
Language:English
Subjects:
bone marrow failure
chemotherapy
Child
Disease Management
Drug Therapy
hematology
Hodgkin Disease - complications
Humans
India
lymphoma
myelofibrosis
Neuroblastoma - complications
Primary Myelofibrosis - diagnosis
Primary Myelofibrosis - drug therapy
Primary Myelofibrosis - etiology
Retroperitoneal Neoplasms - complications
Thrombasthenia - complications
Treatment Outcome
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Summary: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.
ISSN:0888-0018
1521-0669
DOI:10.3109/08880011003739430