Details

Autor(en) / Beteiligte

• Hernández, J.M.
• del Cañizo, M. C.
• Cuneo, A.
• García, J. L.
• Gutiérrez, N. C.
• González, M.
• Castoldi, G.
• San Miguel, J. F.

Titel

Clinical, hematological and cytogenetic characteristics of atypical chronic myeloid leukemia

Ist Teil von

• Annals of oncology, 2000-04, Vol.11 (4), p.441-444

Ort / Verlag

Oxford: Oxford University Press

Erscheinungsjahr

2000

Quelle

MEDLINE

Beschreibungen/Notizen

• 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 × 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.