title: Agnogenic myelofibrosis in children--a case report and review of the literature.
abstract: The case presented here is an 18-month-old female infant with agnogenic myelofibrosis. Clinically, this case was characterized by pancytopenia, hepatosplenomegaly, and rapidly fatal course, without response to steroids or other chemotherapy. Bone marrow biopsy and postmortem examination, revealed myeloid metaplasia of the liver, spleen, and lymph nodes, and conspicuous proliferation of immature cells, presumably of reticulum cells or precursors of hematopoietic cell lines, associated with prominent proliferation of reticular fibrous tissue. These morphologic features corresponded with those of agnogenic myelofibrosis, classified as a form of myeloproliferative disorder. A review of 15 cases of myelofibrosis in children collected from the literature showed a mean age of 2 years and 9 months with most cases being less than 2 years of age. There was a distinct female preponderance, while male infants with myelofibrosis were associated with Down's syndrome or C monosomy. The mean survival was 6.7 months. Approximately 25 cases of myelofibrosis have been reported in the English literature, while only 5 cases have been described in Japan.
mesh_terms: D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008854:Microscopy, Electron; D055728:Primary Myelofibrosis / Q000209:etiology / Q000473:pathology*