<症例>Terminal deoxynucleotidyl transferase陽性の巨核芽球性白血病

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A case of terminal deoxynucleotidyl transferase (TdT) positive megakaryoblastic leukemia is reported. A thirty-three year old male was presented to the Kinki University Hospital because of palpitation and general fatigue. A diagnosis of megakaryoblastic leukemia with pancytopenia, bone marrow fibrosis and hepatosplenomegaly was made. He was started on the therapy of prednisolone. The blasts of peripheral blood were decreased after the therapy, but they were increased again. The different treatment consisting of the therapies with prednisolone-vincristine, VP-16 and Ara-c low dose also could not obtain the complete remission. He died of pulmonary mycosis 22 months after the first admission. His hematologic and bone marrow pictures were unchanged. The cells of peripheral blast had a round or irregular round nucleus with minute nuclear net structure and a few nucleoli, having a poor cytoplasm with a portion of granule. Cytochemical findings showed the case to be strongly positive in acid phosphatase, positive in α-naphtyl acetate esterase (α-NAE), and negative in peroxidase, PAS and α-naphtyl butyrate esterase (α-NBE). The bone marrow aspiration showed dry tap and histological examination of the bone marrow picture from the biopsy specimen revealed marked hyperplasia of cells and fibrosis. Electron microscopic picture of the blast cells demonstrated that the case had no α-granules and platelet demarcation membrane. Platelet-peroxidase reaction was positive in endoplasmic reticulum and perinuclear envelope cisternae of the blast cells but was negative in granules and Golgi apparatus. The karyotype was 46,XY, 20q-.
近畿大学の論文
1988-06-25

<症例>Terminal deoxynucleotidyl transferase陽性の巨核芽球性白血病

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