Two patients with Ph1-positive chronic myelogenous leukemia with pleural blastic transformation occurring before medullary involvement are presented. The clonal origin of the pleural cells identified as unclassified blasts in 1 patient and as erythroid blasts in the other was confirmed by the presence of the t(9;22) translocation and their clonal evolution by the presence of duplicated Ph1 and additional chromosome alterations. DNA obtained from the pleural blasts and peripheral blood cells of 1 patient showed an identically rearranged bcr configuration, indicating the origin of the pleural blasts from the CML clone and suggesting that this genomic event is not directly linked with the progression of disease.