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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.

Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion
|October 22, 2014
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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.

Keywords:
IdiopathicMyelofibrosisPediatric

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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.