Two autopsied cases of Philadelphia chromosome (Ph
1)-positive acute myelogenous leukemia (AML) are reported, and the pertinent literature is reviewed. The cases, a 69-year old Japanese male and a 60-year-old Irishman, were admitted for further examination of leukocytosis.
On admission, either marked splenomegaly or basophilia was not found, while the proliferation of blasts in peripheral blood and bone marrow was remarkable. These blasts were positive to peroxidase and sudan black B staining. Neutrophile alkaline phosphatase score remained within normal range in one patient and slightly decreased in another.
Chromosomal analyses of bone marrow cells showed hypodiploidy with Ph
1 in almost all metaphases, and the karyotypes were [45, XY, -7, t (9; 22)] and [45, XY, -18, t (9; 22)], respectively. It might be suggested that both of them could be diagnosed as Ph
1-positive AML, whereas these monosomies were very rare in the blastic crisis of chronic myelogenous leukemia (CML).
One survived for 4 months instead of no response to therapy, while 10 weeks' complete remission was induced in the other by combination chemotherapy consisting of vincristin, cytosine arabinoside, 6-mercaptopurine and prednisolone. During the remission, no characteristic features of chronic phase in CML were observed. The normal karyotype could not be identified during the remission, because of no bone marrow cells in metaphase. He survived for 7 months after diagnosis.
From 31 cases of Ph
1-positive AML identified by banding analysis in the literature some characteristic features were deduced as follows; 1) lack of marked splenomegaly, 2) lack of basophilia, 3) mosaic consisting of Ph
1-positive and Ph
1-negative cells, 4) occasional disappearance of Ph
1-positive cells during remission, 5) high incidence of an unusual translocation of Ph
1, and 6) characteristic additional chromosomal aberrations.
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