Paediatrics primary myelofibrosis and acute stroke: A rare presentation

Area of Science:

• Hematology
• Pediatric Oncology
• Pathology

Background:

• Idiopathic myelofibrosis (IMF) is a rare myeloproliferative neoplasm characterized by bone marrow fibrosis.
• Pediatric primary myelofibrosis is exceptionally rare, posing diagnostic and therapeutic challenges.

Observation:

• A 6-year-old male presented with pallor, petechiae, and gingival bleeding.
• Bone marrow aspirate revealed scattered erythroid and myeloid precursors.
• Trephine biopsy showed significant fibrotic activity and histiocyte clusters.

Findings:

• The patient was diagnosed with pediatric primary myelofibrosis based on bone marrow biopsy findings.
• The patient subsequently experienced a stroke, leading to left-sided hemiparesis.
• MRI confirmed an intraparenchymal hemorrhage and meningeal extramedullary hematopoiesis.

Implications:

• This case underscores the rarity and complex presentation of pediatric primary myelofibrosis.
• The occurrence of stroke and extramedullary hematopoiesis highlights potential serious complications.
• Further research into pediatric myelofibrosis is warranted to improve understanding and management.