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RA Streuli, JR Testa, JW Vardiman, U Mintz, HM Golomb and JD Rowley
Clinical and cytogenetic studies were done on 8 patients with
dysmyelopoietic syndrome: 6 of these patients had refractory anemia with an
excess of blasts (RAEB), and 2 patients had chronic myelomonocytic leukemia
(CMML) according to the French-American-British classification. The ages of
these 8 patients (3 female and 5 male) ranged from 45 to 70 yr (median,
61.5 yr). Seven of the 8 patients died 3-86 mo (median, 11 mo) after the
onset of symptoms of hemorrhage or infections. Cytogenetic studies of bone
marrow cells with the Q-banding technique showed clonal karyotypic
abnormalities in 7 of the 8 patients (87.5%). Five of the 7 chromosomally
abnormal patients had very complex karyotypes; all 7 patients, however, had
at least 1 of 4 specific changes: -5 (or 5Q-), -7, +8, and +21. Three of
the 7 patients with abnormal karyotypes had had some exposure to potential
mutagenic/carcinogenic agents. Five of the 7 patients had serial
cytogenetic analyses, 4 of which showed evolution of the karyotype to
further complexity; in 2 cases, this coincided with the evolution of the
disease into acute leukemia. The median survival time of patients whose
initial cytogenetic samples showed both normal and abnormal metaphases was
more than twice that of patients who had only abnormal metaphases initially
(12 mo versus 4.5 mo).
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