A 73-year-old woman developed a rapidly fatal disease that fit the clinical criteria for acute myelofibrosis. Over a 9 month period she progressed from normal peripheral blood counts to severe pancytopenia and finally a terminal phase with monocytosis and circulating myeloblasts. Morphologic examination of her bone marrow at presentation showed trilineage dysplasia, hypercellularity, and diffuse fibrosis with foci of immature precursors. Cytogenetic, analysis showed the karyotype 45-46, XX,del(5)(q33),+11,add(17)(q25),-18,-20,+22. The morphologic and cytogenetic findings in this case support a relationship between acute myelofibrosis and myelodysplastic syndromes. Acute myelofibrosis with complex chromosomal aberrations may represent one pathway of evolution in myelodysplasia that is associated with a particularly poor prognosis.
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