Abstract
A 45-year-old man admitted on December 1, 1980 because of easy fatigability and high fever. On admission, he had hepatosplenomegaly, lymphadenopathy and scattered petechae. Hematologic examination showed a moderate anemia, a white cell count of 222,000/mm3 with 94.5% lymphoblasts and marked thrombocytopenia. Bone marrow aspiration revealed 98% lymphoblasts. Their surface marker study yielded E rosettes formation in 2% and EAC rosettes formation in 2%. The terminal deoxynucleotidyl transferase activity by the immunofluorescent assay was elevated. The karyotype of the bone marrow cells was 46, XY, t(9q+; 22q-). He was diagnosed as haaving Ph1 positive acute lymphoblastic leukemia or lymphoid blast crisis at the onset of chronic myelogenous leukemia (CML). He was treated with viencristine, Endoxan, 6MP and prednisolone (VEMP), and a complete remission was achieved 3 months after admission. Subsequently, the peripheral blood showed changes similar to those in the chronic phase of CML. In April, 1981, the number of lymphoblasts in the peripheral blood and in the bone marrow rapidly increased and the patient was readmitted. He became febrile with septecemia due to E. coli and Pseudomonas, and died on June 30, 1981.
At autopsy, infiltration of lymphoid cells was seen in the bone marrow, spleen, liver, kidneys, lymph nodes, lungs, and protoate. The past reports on the cases with CML presenting as acute leukemia at onset and Ph1 positive acute leukemia have been reviewed.
