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000148858 041__ $$aeng
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000148858 1001_ $$aChapiro, Elise$$b0
000148858 245__ $$aGenetic characterization of B-cell prolymphocytic leukemia: a prognostic model involving MYC and TP53.
000148858 260__ $$aStanford, Calif.$$bHighWire Press$$c2019
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000148858 520__ $$aB-cell prolymphocytic leukemia (B-PLL) is a rare hematological disorder whose underlying oncogenic mechanisms are poorly understood. Our cytogenetic and molecular assessments of 34 patients with B-PLL revealed several disease-specific features and potential therapeutic targets. The karyotype was complex (≥3 abnormalities) in 73% of the patients and highly complex (≥5 abnormalities) in 45%. The most frequent chromosomal aberrations were translocations involving MYC [t(MYC)] (62%), deletion (del)17p (38%), trisomy (tri)18 (30%), del13q (29%), tri3 (24%), tri12 (24%), and del8p (23%). Twenty-six (76%) of the 34 patients exhibited an MYC aberration, resulting from mutually exclusive translocations or gains. Whole-exome sequencing revealed frequent mutations in TP53, MYD88, BCOR, MYC, SF3B1, SETD2, CHD2, CXCR4, and BCLAF1. The majority of B-PLL used the IGHV3 or IGHV4 subgroups (89%) and displayed significantly mutated IGHV genes (79%). We identified 3 distinct cytogenetic risk groups: low risk (no MYC aberration), intermediate risk (MYC aberration but no del17p), and high risk (MYC aberration and del17p) (P = .0006). In vitro drug response profiling revealed that the combination of a B-cell receptor or BCL2 inhibitor with OTX015 (a bromodomain and extra-terminal motif inhibitor targeting MYC) was associated with significantly lower viability of B-PLL cells harboring a t(MYC). We concluded that cytogenetic analysis is a useful diagnostic and prognostic tool in B-PLL. Targeting MYC may be a useful treatment option in this disease.
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000148858 7001_ $$aPramil, Elodie$$b1
000148858 7001_ $$aDiop, M'boyba$$b2
000148858 7001_ $$aRoos-Weil, Damien$$b3
000148858 7001_ $$aDillard, Clémentine$$b4
000148858 7001_ $$aGabillaud, Clémentine$$b5
000148858 7001_ $$aMaloum, Karim$$b6
000148858 7001_ $$aSettegrana, Catherine$$b7
000148858 7001_ $$aBaseggio, Lucile$$b8
000148858 7001_ $$aLesesve, Jean-François$$b9
000148858 7001_ $$aYon, Mélanie$$b10
000148858 7001_ $$aJondreville, Ludovic$$b11
000148858 7001_ $$aLesty, Claude$$b12
000148858 7001_ $$aDavi, Frédéric$$b13
000148858 7001_ $$aLe Garff-Tavernier, Magali$$b14
000148858 7001_ $$aDroin, Nathalie$$b15
000148858 7001_ $$aDessen, Philippe$$b16
000148858 7001_ $$aAlgrin, Caroline$$b17
000148858 7001_ $$aLeblond, Véronique$$b18
000148858 7001_ $$aGabarre, Jean$$b19
000148858 7001_ $$aBouzy, Simon$$b20
000148858 7001_ $$aEclache, Virginie$$b21
000148858 7001_ $$aGaillard, Baptiste$$b22
000148858 7001_ $$aCallet-Bauchu, Evelyne$$b23
000148858 7001_ $$aMuller, Marc$$b24
000148858 7001_ $$aLefebvre, Christine$$b25
000148858 7001_ $$aNadal, Nathalie$$b26
000148858 7001_ $$aIttel, Antoine$$b27
000148858 7001_ $$aStruski, Stéphanie$$b28
000148858 7001_ $$aCollonge-Rame, Marie-Agnès$$b29
000148858 7001_ $$aQuilichini, Benoit$$b30
000148858 7001_ $$aFert-Ferrer, Sandra$$b31
000148858 7001_ $$aAuger, Nathalie$$b32
000148858 7001_ $$aRadford-Weiss, Isabelle$$b33
000148858 7001_ $$aWagner, Lena$$b34
000148858 7001_ $$0P:(DE-He78)648d23797edea029cdadba96810a9e8c$$aScheinost, Sebastian$$b35$$udkfz
000148858 7001_ $$aZenz, Thorsten$$b36
000148858 7001_ $$aSusin, Santos A$$b37
000148858 7001_ $$aBernard, Olivier A$$b38
000148858 7001_ $$aNguyen-Khac, Florence$$b39
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