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Title

Natural history and cell of origin of TCF3-ZNF384 and PTPN11 mutations in monozygotic twins with concordant BCP-ALL

AuthorsBueno, Clara; Tejedor, Juan Ramón; Bashford-Rogers, Rachael; González-Silva, Laura; Valdés-Mas, Rafael; Agraz-Doblas, Antonio; Díaz de la Guardia, Rafael; Ribera, Jordi; Zamora, Lurdes; Bilhou-Nabera, Chrystele; Abermil, Nassera; Guermouche, Hélène; Gouache, Elodie; Leverger, Guy; Fraga, Mario F.; Fernández, Agustín F.; Ballerini, Paola; Varela, Ignacio ; Menéndez, Pablo
Issue Date2019
PublisherAmerican Society of Hematology
CitationBlood 134(11): 900-905 (2019)
AbstractTo the editor: B-cell precursor acute lymphoblastic leukemia (BCP-ALL) is the most common childhood cancer and is molecularly heterogeneous. Identical twins with concordant BCP-ALL have provided a unique and tractable model to delineate the natural history and clonal evolution of the disease. Such studies have led to the 2-hit model for pediatric BCP-ALL, elucidated by Greaves, which provides unambiguous evidence that an initiating alteration, often occurring in utero, generates a preleukemic clone, which eventually gives rise to an overt leukemia upon acquisition of secondary cooperating mutations. Importantly, genome-wide sequencing has revealed a strikingly silent mutational landscape in childhood BCP-ALL, further indicative of a developmental cancer of prenatal origin, with a short window of time to accumulate molecular alterations.
Publisher version (URL)https://doi.org/10.1182/blood.2019000893
URIhttp://hdl.handle.net/10261/201812
DOI10.1182/blood.2019000893
ISSN0006-4971
E-ISSN1528-0020
Appears in Collections:(IBBTEC) Artículos
(CINN) Artículos
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