Ediatric Hyperfibrotic Myelodysplasia: An Unusual Clinicopathologic Entity

Abstract

This study is presented to highlight a rare pediatric bone marrow pathology in which features of bone marrow dysplasia coexist with severe fibrosis in a clinical setting of fever, anemia, and organomegaly. Fourteen children (nine males, five females) clinically presented with fever, anemia, and hepatosplenomegaly. Extensive bone marrow fibrosis with dysplastic features was seen in their marrow precursor cells. Peripheral blood smears showed teardrop poikilocytes, thrombocytopenia, and occasional blasts. In conclusion, pediatric hyperfibrotic myelodysplasia is a distinct clinicopathologic entity. Myeloproliferative and dysmyelopoietic syndromes are complex disorders that are interrelated and not always easily diagnosed only on morphologic grounds.

Key Words:

• hyperfibrotic
• myelodysplasia
• pediatric