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Paediatric idiopathic myelofibrosis
Annapurna Saksena1, Prerna Arora1, Nita Khurana1
1Department of Pathology, Maulana Azad Medical College, Bahadur Shah Zafar Marg, New Delhi, 110002 Delhi India.
Summary
Idiopathic pediatric myelofibrosis is a rare condition in children, often presenting with enlarged spleen and liver. Diagnosis requires excluding secondary causes and confirming marrow fibrosis.
Area of Science:
- Hematology
- Pediatric Oncology
Background:
- Pediatric myelofibrosis is a rare hematologic disorder.
- It is typically secondary to other conditions, but can be idiopathic.
- Idiopathic myelofibrosis is exceptionally rare in children.
Observation:
- A case of idiopathic myelofibrosis in a 10-year-old male is presented.
- Bone marrow aspirate was dilute, and bone biopsy revealed marrow fibrosis (grade 2-3 reticulin fibers).
- Iliac lymph node biopsy showed reactive sinus histiocytosis with extramedullary hematopoiesis.
Findings:
- The patient was diagnosed with idiopathic pediatric primary myelofibrosis.
- Exclusion of granuloma, parasites, and infiltrative disorders was confirmed.
- Acid-fast bacillus stain was negative.
Implications:
- This case highlights the importance of considering idiopathic pediatric myelofibrosis.
- Suspect this diagnosis in children with progressive pallor, hepatosplenomegaly, and a dry bone marrow tap.
- Bone marrow biopsy confirming fibrosis is crucial after excluding secondary causes.
