Haema 2012; 3(1): 2-9
by Georgios Georgiou1, Photis Beris1,2
1Unilabs, Département d’Hématologie, Coppet Switzerland; 2Département de Médecine Interne, Faculté de Médecine, Université de Genève, Genève Suisse
Abstract
Chronic myelogenous leukemia was most probably described for the fist time in 1845. The establishment however of myeloproliferative disorders as a separate entity, belongs to William Dameshek in 1951 who is considered officially the haematologist that classified CML in haematologic malignan- cies outside acute leukemias. In 1960 a chromosomal abnormality was observed for the first time and the name Philadelphia chromosome was coined due to the city where it was described. Janet Rowley, from the USA, in 1972 described for the first time the translocation t(9;22). Ten years later the human homolog of vabl was isolated and in relatively short period (1984) researchers showed that the break point in chromosome 22 was always occurred in relatively small region named bcr. Finally at the beginning of 1990 the oncogenic effect of bcrabl was demonstrated by the induction of the disease in mouse. At the beginning, treatment of CML was palliative and the disease was considered incurable. Busulfan was the first cytostatic which relatively easier than radiation, controls extreme leucocytosis which with- out any therapeutic intervention was always fatal. Hydroxyurea was introduced in therapeutics in 1960 and was and still is, the best drug to control leucocytosis without acceleration of the disease towards its acute phase. However hydroxyurea can not modify the physical evolution of the disease. The introdution of autologous bone marrow transplantation in medicine was the first hope to perpetuate the disease in its chronic phase, fact that it was never achieved by any investigator. The first real hope to prolong the chronic phase of the disease was interferon – α, which achieved a significant increase of overall survival of patients for 1 – 2 years as compared to hydroxyurea. Association of interferon – α with AraC was considered the goal standard of treatment of CML before the era of tyrosine kinase inhibitors.