Annals of Oncology 11:441-444, 2000
© 2000 European Society for Medical Oncology
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Clinical, hematological and cytogenetic characteristics of atypical chronic myeloid leukemia
1Departments of Hematology, Hospital Universitario and Centro de Investigation del Cáncer, Universidad de Salamanca-CSIC Spain
2University of Ferrara Italy
Correspondence to: J. M. Hernández, MD, Servicio de Hematologia, Hospital Universitario de Salamanca, Paseo San Vicente 58-182, 37007 Salamanca, Spain, E-mail: jmhernandezr{at}aehh.org
Background: Atypical chronic myeloid leukemia (aCML) is an infrequent chronic myeloproliferative disorder characterized by leukocytosis, absence of Philadelphia chromosome or BCR-ABL rearrangement, and marked myeloid dysplasia. Some cases have an absolute monocytosis but can be distinguished from chronic myelomonocytic leukemia (CMML) by the presence of a higher percentage (> 15%) of circulating immature granulocytes.
Patients and methods: In a series of 11 patients with a diagnosis of aCML according to the FAB proposals we have analyzed the most relevant clinical, hematological and cytogenetic characteristics.
Results: The median age was 65 years (16–84). All but one case showed, at time of diagnosis, leukocytosis (median WBC was 36 x 109/l), 55% had moderate anemia and 36% had thrombocytopenia. Most cases had marked dysplasia, particularly in the granulocytic lineage (82% of the cases), and all cases showed bone marrow red hypoplasia. Cytogenetic abnormalities were present in 9 out of the 11 patients. Trisomy 8 was observed in three cases and other clonal chromosomal abnormalities included deletions of 5q, 13q, 17p, 12q, and llq as well as a t(6;8)(p23;q22) translocation. Fluorescence in situ hybridization (FISH) studies failed to demonstrate ETV-6 gene involvement. The median survival time from diagnosis was only 14 months (range 3–56 months).
Conclusions: These data suggest that a CML is a rare disease which is characterized by leukocytosis, with dysgranulo-poiesis, BM erythroid hypoplasia, chromosomal, though not recurrent, abnormalities and poor prognosis.
aCML, CMML, cytogenetics, FISH, MDS, Philadelphia-negative CML
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