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Atypical Chronic Myeloid Leukaemia

Atypical Chronic Myeloid Leukaemia (aCML) is a leukemic disorder having both myelodysplastic and myeloproliferative features.1 It differs from classic Chronic Myeloid Leukaemia (CML) in 2 important ways: First, whereas the vast majority of cases of CML are associated with the t(9;22)(q43;q11.2) chromosomal translocation (Ph chromosome) and Bcr-Abl fusion gene, aCML is not.2,3 Also, unlike what is seen in the chronic phase of CML, aCML is associated with marked granulocytic and frequently multilineage dysplasia.3

 

Epidemiology

The incidence of aCML is unknown. The median age at the time of diagnosis has been reported to be between 70 and 80 years;1 however, the disorder can be found in middleaged patients as well.4

References:

1. National Cancer Institute. Myelodysplastic/Myeloproliferative Diseases (PDQ®): Treatment. Health Professional Version. Available at: http://www.cancer.gov/cancertopics/pdq/treatment /mds-mpd/healthprofessional/allpages. Accessed February 3, 2009.

2. Goldman JM, Melo JV. Chronic myeloid leukaemia—advances in biology and new approaches to treatment. N Engl J Med. 2003;349:1451-1464.

3. Vardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neoplasms. Blood. 2002;100:2292-2302.

4. Hernandez JM, del Canizo MC, Cuneo A, et al. Clinical, hematological and cytogenetic characteristics of atypical chronic myeloid leukaemia. Ann Oncol. 2000;11:441-444.