Myelofibrosis in children: experience at a single tertiary care center in India
| Autor: | Ketan Prasad Kulkarni, Rajive Kumar, D Chandra, Laxman Singh Arya, Ramesh Dawar, Vasantha Thavraj |
|---|---|
| Rok vydání: | 2010 |
| Předmět: |
Pediatrics
medicine.medical_specialty Hepatosplenomegaly India Pallor Neuroblastoma Pharmacotherapy Drug Therapy Internal medicine medicine Humans Retroperitoneal Neoplasms Disease management (health) Myelofibrosis Child Hematology business.industry Bone marrow failure Disease Management medicine.disease Hodgkin Disease Surgery Lymphoma Treatment Outcome Oncology Primary Myelofibrosis Pediatrics Perinatology and Child Health medicine.symptom business Thrombasthenia |
| Zdroj: | Pediatric hematology and oncology. 27(5) |
| ISSN: | 1521-0669 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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