Pediatric Bone Marrow Myelofibrosis: A Heterogeneous “Entity” Requiring Refined Classification to Guide Therapy

Diagnosing and treating cytopenic myelofibrosis in children is challenging due to the wide spectrum of clinical and pathological features, underlying etiologies, and variable therapeutic responses. In this review, we summarize the related literature and present our diagnostic algorithm to differentiate pediatric myelofibrosis and guide therapy. In brief, primary myelofibrosis is extremely rare in children, while myelofibrosis secondary to non-neoplastic or neoplastic disorders should be thoroughly ruled out in ambiguous cases. Moreover, it is reasonable to closely follow up patients and repeat bone marrow biopsy before reaching a definitive diagnosis.