title: Chronic Childhood Idiopathic Myelofibrosis in Down's Syndrome: A Case Report.
abstract: Idiopathic myelofibrosis a disease of elderly is rarely seen in children. A case of chronic idiopathic myelofibrosis in an 8 year old boy with Down's syndrome is reported here, who presented with progressive pallor and hepatosplenomegaly. Peripheral blood examination revealed pancytopenia, macrocytic anemia and tear drop cells. No blasts were found. Bone marrow aspirate yielded a dry tap and trephine biopsy showed marrow fibrosis with osteosclerosis. Focally megakaryocytes were increased in number with atypical morphology. No blasts were seen. Review of literature revealed 47 reported cases of childhood idiopathic myelofibrosis. Six cases were associated with Down's syndrome and only 3 of them had features of chronic idiopathic myelofibrosis without evidence of acute megakaryoblastic leukaemia.
authors.affiliation: Department of Pathology, Lady Hardinge Medical College & Associated Kalawati Saran Children Hospital, A-207, Narwana Appartments, Plot no. 89, I.P. Extension, New Delhi, 110092 India.
authors.forename: Smita
authors.initials: S
authors.lastname: Singh
authors.forename: Sunita
authors.initials: S
authors.lastname: Sharma
authors.forename: Narender
authors.initials: N
authors.lastname: Tejwani
authors.forename: Jagdish
authors.initials: J
authors.lastname: Chandra
keywords: Childhood; Down’s syndrome; Idiopathic myelofibrosis; Osteosclerosis