Chronic myelogenous leukemia: Prolonged survival with spontaneous decline in the frequency of Ph1-positive cells and subsequent development of mixed Ph1-positive and Ph1-negative blast crisis
✍ Scribed by Frederick R. Appelbaum; Vesna Najfeld; Jack W. Singer
- Publisher
- John Wiley and Sons
- Year
- 1983
- Tongue
- English
- Weight
- 486 KB
- Volume
- 51
- Category
- Article
- ISSN
- 0008-543X
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✦ Synopsis
A 23-year-old man developed Ph'-positive chronic myelogenous leukemia in 1966. After two short courses of busulfan not associated with severe myelosuppression, the frequency of Ph'-positive metaphases in his bone marrow was 40%. Over the next eight years, he remained hematologically stable without further therapy. During that time there was a progressive decline in the frequency of Ph'positive metaphases in his bone marrow to 3% by 1976. His disease subsequently transformed to an acute lymphoblastic leukemia. Cytogenetic studies of the marrow at the onset of blast crisis and of cultured marrow blast cells suggested that the blasts were a mixture of Ph'-positive and Ph'-negative cells. This patient was thus unique in that he demonstrated spontaneous decline in the frequency of Ph'-positive cells in his bone marrow and he developed blast crisis with an apparent mixture of Ph'positive and Ph'-negative blasts. These findings demonstrate that a Ph'-positive cell clone may lose its proliferative advantage over Ph'-negative cells and raise the possibility that Ph'-negative cells persisting in patients with Ph'-positive chronic myelogenous leukemia may be abnormal and, like the Ph'-positive cells, may be susceptible to acute leukemic transformation.