Clinical, hematological and cytogenetic characteristics of atypical chronic myeloid leukemia.

Abstract

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 10(9)/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 11q 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 aCML is a rare disease which is characterized by leukocytosis, with dysgranulopoiesis, BM erythroid hypoplasia, chromosomal, though not recurrent, abnormalities and poor prognosis.

• Authors: J M Hernández, M C del Cañizo, A Cuneo, J L García, N C Gutiérrez, M González, G Castoldi, J F San Miguel
• Journal: Annals of oncology : official journal of the European Society for Medical Oncology (Ann Oncol) Vol. 11 Issue 4 Pg. 441-4 (Apr 2000) ISSN: 0923-7534 [Print] England
• PMID: 10847463 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adolescent
• Aged
• Anemia
• Bone Marrow Cells (pathology)
• Chromosome Aberrations
• Female
• Humans
• In Situ Hybridization, Fluorescence
• Leukemia, Myelogenous, Chronic, BCR-ABL Positive (classification, genetics, pathology)
• Leukocytosis
• Male
• Middle Aged
• Prognosis
• Survival Analysis
• Thrombocytopenia