Characteristics and clinical significance of cytogenetic abnormalities in polycythemia vera.

Autor:	Tang G; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA gtang@mdanderson.org., Hidalgo Lopez JE; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Wang SA; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Hu S; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Ma J; Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Pierce S; Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Zuo W; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Carballo-Zarate AA; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Yin CC; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Tang Z; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Li S; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Medeiros LJ; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Verstovsek S; Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA., Bueso-Ramos CE; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Jazyk:	angličtina
Zdroj:	Haematologica [Haematologica] 2017 Sep; Vol. 102 (9), pp. 1511-1518. Date of Electronic Publication: 2017 May 04.
DOI:	10.3324/haematol.2017.165795
Abstrakt:	Up to 20% of patients with polycythemia vera have karyotypic abnormalities at the time of the initial diagnosis. However, the cytogenetic abnormalities in polycythemia vera have not been well characterized and their prognostic impact is largely unknown. In this study, we aimed to address these issues using a large cohort of polycythemia vera patients with cytogenetic information available. The study included 422 patients, 271 in polycythemic phase, 112 with post-polycythemic myelofibrosis, 11 in accelerated phase, and 28 in blast phase. Abnormal karyotypes were detected in 139 (33%) patients, ranging from 20% in those in the polycythemic phase to 90% among patients in accelerated/blast phase. Different phases harbored different abnormalities: isolated del(20q), +8 and +9 were the most common abnormalities in the polycythemic phase; del(20q) and +1q were the most common abnormalities in post-polycythemic myelofibrosis; and complex karyotypes were the most common karyotypes in accelerated and blast phases. Patients with an abnormal karyotype showed a higher frequency of disease progression, a shorter transformation-free survival and an inferior overall survival compared with patients with a normal karyotype in the same disease phase. Cytogenetics could be effectively stratified into three risk groups, low- (normal karyotype, sole +8, +9 and other single abnormality), intermediate- (sole del20q, +1q and other two abnormalities), and high-risk (complex karyotype) groups. We conclude that cytogenetic changes in polycythemia vera vary in different phases of disease, and carry different prognostic impacts. (Copyright© 2017 Ferrata Storti Foundation.)
Databáze:	MEDLINE
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