A 42-year-old man with severe pancytopenia and myelofibrosis underwent splenectomy seven months after onset of his symptoms; the leukocyte, platelet, and hematocrit levels became normal. Myeloid metaplasia was identified in the liver and spleen. Progressive lymphocytosis started eight months after splenectomy, and after 66 months a florid hairy-cell leukemia was diagnosed; the circulating cells were B type with micro K surface markers. Anemia and thrombocytopenia reappeared and were controlled initially with daily prednisone; chlorambucil was later added. At that time, the peripheral blood had more than 150 megaloblastoid-appearing normoblasts per 100 leukocytes. The PAS stain was positive in 95% to 100% of these cells; the B-cell surface markers were no longer identified. Further treatment failed to control the lymphoproliferative and myeloproliferative syndromes; the patient died 99 months after splenectomy. On autopsy, infiltration by hairy-cell leukemia cells and erythroid precursors was observed in the bone marrow, liver, lymph nodes, and other organs.