Promyelocytic crisis in chronic myelogenous leukemia transformed to basophilic crisis with clonal evolution during a short period
Fujii, H.; Maekawa, T.; Misawa, S.; Yokota, S.; Urata, Y.
Nihon Ketsueki Gakkai Zasshi Journal of Japan Haematological Society 51(3): 587-595
1988
ISSN/ISBN: 0001-5806 PMID: 3166569 Document Number: 307819
A 50-year-old Japanese female with Ph1-positive CML was diagnosed as having blastic crisis nine years after the onset. At blastic crisis, the white blood cell count was 21, 470/.mu.l with 2% myeloblasts, 5% promyelocytes and 14% basophils. Bone marrow aspirate revealed increased cellularity with 62% promyelocytes, which were positive for peroxidase staining but were not stained metachromatically with toluidine blue. Therefore, she was diagnosed as having promyelocytic crisis and combined therapy consisting of vincristine and prednisolone was administered. Cytogeneic study on bone marrow cells revealed three clones: 46,XX,Ph1, 46,XX,Ph1, i(17q) and 46,XX,Ph1, i(17q), i(18q). In vitro clonal culture of hematopoietic precursors revealed that a blastic clone with an i(17q) chromosome abnormality could differentiate to at least three cell lineages involving neutrophil, eosinophil and basophil. One month later, hematological findings showed marked basophilia in peripheral blood (56%), as well as in bone marrow (42%). Most basophils were morphologically immature, stained metachromatically with toluidine blue and were positive for PAS staining. Electron microscopic examination showed a number of granules and lamellar structures that were specific for basophils and mast cells, respectively. Cytogenetic study on bone marrow cells at the phase of basophilic crisis revealed three types of metaphases with a clonal evolution of 46,XX,Ph1,i(17q), 46,XX,Ph1,i(17q),i(18q) and 46,XX,Ph1,i(17q), +6. After 2 weeks, she died of bronchopneumonia despite therapy. Autopsy revealed evidence of disseminated intravascular coagulopathy in renal glomeruli.