著明な好酸球増多を呈したMonosomy-7 Myeloproliferative Disorderの1例

抄録

A case of monosomy-7 myeloproliferative disorder is reported. A 13-year-old boy was admitted with fever. He had no hepatosplenomegaly. The peripheral blood showed a mild anemia and leukocytosis with 75% blasts. Bone marrow aspiration revealed a normal cellularity with 23.1% peroxidase-negative blasts. Initially he was diagnosed to have ALL and received a combination chemotherapy of vincristine and prednisolone without success. The number of blasts decreased markedly after the chemotherapy was stopped.
The cytogenetic analysis of bone marrow cells showed a karyotype of 45, XY, -7. Therefore, the diagnosis was changed to monosomy-7 myeloproliferative disorder. The same karyotypic abnormality was found a month later. Subsequently, marked eosinophilia and leukemic cell infiltration into the skin and mandibular region were seen. In the terminal stage, progression to AML occurred as evidenced by peroxidase-positive blasts.