Pediatric Myelofibrosis: State of the Art

Bone marrow fibrosis (BMF) has a multifactorial basis that includes benign (infective, autoimmune and/or autoinflammatory, metabolic/endocrine), iatrogenic, as well as malignant etiologies. Therefore, careful evaluation of BMF requires a systematic approach incorporating morphological, immunophenotypic, serological/immunological, as well as molecular tools to reach at a conclusive diagnosis before initiating definitive therapy. While most of the conditions associated with BMF are common in adults, occurrence of adult-like BMF in pediatric and/or adolescent age group is uncommonly or sporadically reported. Specific entities like pediatric immune myelofibrosis (pedIMF), myeloid-myeloproliferative-myelodysplastic neoplasm associated fibrosis, and other rarer entities essentially require next generation sequencing (NGS) or whole exome sequencing (WES) based tools for early and accurate diagnosis. This comprehensive review aims to highlight the recent concepts in pediatric BMF from a hematopathological perspective to create enhanced awareness for better diagnosis and effective patient care.