IDIOPATHIC MYELOFIBROSIS IN CHILDREN

Childhood myelofibrosis (Mf) is rare with variable outcome reported in the literature. We present clinical and investigative details of thre e children who presented with idiopathic Mf in early childhood, Two of these children were identical twins and have been haematologically st able over the past 7 years since their diagnosis. The third patient un derwent an allogeneic bone marrow transplant (BMT) procedure as her cl inical status was deteriorating. She remains engrafted at 13 months po st BMT. None of the children had hepatosplenomegaly although extramedu llary haemopoiesis was demonstrated on Fe-57 studies in two patients. Circulating progenitors in these patients were increased in the face o f reduced marrow precursors. The aetiology of childhood Mf is unclear and its natural history seems different from the adult disease. Alloge neic BMT can be an option for definitive treatment.