TY - JOUR
T1 - Chronic lymphocytic leukemia and prolymphocytic leukemia with MYC translocations
T2 - A subgroup with an aggressive disease course
AU - Put, Natalie
AU - Van Roosbroeck, Katrien
AU - Konings, Peter
AU - Meeus, Peter
AU - Brusselmans, Caroline
AU - Rack, Katrina
AU - Gervais, Carine
AU - Nguyen-Khac, Florence
AU - Chapiro, Elise
AU - Radford-Weiss, Isabelle
AU - Struski, Stéphanie
AU - Dastugue, Nicole
AU - Gachard, Nathalie
AU - Lefebvre, Christine
AU - Barin, Carole
AU - Eclache, Virginie
AU - Fert-Ferrer, Sandra
AU - Laibe, Sophy
AU - Mozziconacci, Marie Joëlle
AU - Quilichini, Benoît
AU - Poirel, Hélène A.
AU - Wlodarska, Iwona
AU - Hagemeijer, Anne
AU - Moreau, Yves
AU - Vandenberghe, Peter
AU - Michaux, Lucienne
N1 - Funding Information:
Funding This work was supported by Stichting tegen Kanker. N. Put is supported by Fonds voor Wetenschappelijk Onderzoek (FWO) Vlaanderen. P. Konings and Y. Moreau are supported by grants Katholieke Universiteit Leuven (KUL) Geconcerteerde Onderzoeksacties Mathematical engineering tools for Networks (GOA MaNet), KUL Center of Excellence Symbiosys–KUL Center for Computational Systems Biology (CoE EF/05/007), Belgian Science Policy Interuniversitaire Attractiepolen P6/25 (BelSPO IUAP P6/25), Agentschap voor Innovatie door Wetenschap en Technologie (IWT) Strategisch Basisonderzoek–Molecular Karyotyping (SBO-MoKA), Federale Overheidsdienst (FOD)–Cancer. P. Vandenberghe is a senior clinical investigator of FWO Vlaanderen.
PY - 2012/6
Y1 - 2012/6
N2 - Translocations involving MYC are rare in chronic lymphocytic leukemia (CLL), and up to now, their prognostic significance remains unclear. We report the characteristics of 21 patients with CLL and nine patients with prolymphocytic leukemia (PLL), diagnosed in multiple centers (n=13), which showed an MYC translocation demonstrated by fluorescence in situ hybridization. The prevalence was estimated to be <1%. Advanced age and male predominance were observed. Morphological analysis frequently revealed the presence of prolymphocytes. A typical "CLL-immunophenotype" was found in four of nine cases with PLL. Moreover, CD5 and CD23 were frequently expressed in PLL. The latter findings are atypical for PLL and may suggest transformation or progression of an underlying CLL. MYC translocations were frequently observed with concomitant adverse cytogenetic markers, such as del(11q) (n=8/30) and/or del (17p)/monosomy 17 (n=7/30). In addition, the presence of unbalanced translocations (n=24 in 13/30 cases) and complex karyotype (n=16/30) were frequent in cases with MYC translocations. Altogether, del(17p)/monosomy 17, del(11q), and/or complex karyotype were observed in 22 of 30 patients. Survival outcome was poor: the median time to treatment was only 5 months, and overall survival (OS) from clinical diagnosis and from genetic detection was 71 and 19 months, respectively. In conclusion, CLL/PLL with MYC translocations is a rare entity, which seems to be associated with adverse prognostic features and unfavorable outcome.
AB - Translocations involving MYC are rare in chronic lymphocytic leukemia (CLL), and up to now, their prognostic significance remains unclear. We report the characteristics of 21 patients with CLL and nine patients with prolymphocytic leukemia (PLL), diagnosed in multiple centers (n=13), which showed an MYC translocation demonstrated by fluorescence in situ hybridization. The prevalence was estimated to be <1%. Advanced age and male predominance were observed. Morphological analysis frequently revealed the presence of prolymphocytes. A typical "CLL-immunophenotype" was found in four of nine cases with PLL. Moreover, CD5 and CD23 were frequently expressed in PLL. The latter findings are atypical for PLL and may suggest transformation or progression of an underlying CLL. MYC translocations were frequently observed with concomitant adverse cytogenetic markers, such as del(11q) (n=8/30) and/or del (17p)/monosomy 17 (n=7/30). In addition, the presence of unbalanced translocations (n=24 in 13/30 cases) and complex karyotype (n=16/30) were frequent in cases with MYC translocations. Altogether, del(17p)/monosomy 17, del(11q), and/or complex karyotype were observed in 22 of 30 patients. Survival outcome was poor: the median time to treatment was only 5 months, and overall survival (OS) from clinical diagnosis and from genetic detection was 71 and 19 months, respectively. In conclusion, CLL/PLL with MYC translocations is a rare entity, which seems to be associated with adverse prognostic features and unfavorable outcome.
KW - Burkitt
KW - Chronic lymphocytic leukemia
KW - MYC
KW - Prognosis
KW - Translocation
KW - t(8;14)
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UR - http://www.scopus.com/inward/citedby.url?scp=84862651952&partnerID=8YFLogxK
U2 - 10.1007/s00277-011-1393-y
DO - 10.1007/s00277-011-1393-y
M3 - Article
C2 - 22205151
AN - SCOPUS:84862651952
SN - 0939-5555
VL - 91
SP - 863
EP - 873
JO - Annals of Hematology
JF - Annals of Hematology
IS - 6
ER -