The variable biological signature of refractory cytopenia of childhood (RCC), a retrospective EWOG-MDS study. (September 2021)

• Record Type: Journal Article
• Authors: de Winter, Demi T.C.; Langerak, Anton W.; te Marvelde, Jeroen; Dworzak, Michael N.; De Moerloose, Barbara; Starý, Jan; Locatelli, Franco; Hasle, Henrik; de Vries, Andrica C.H.; Schmugge, Markus; Niemeyer, Charlotte M.; van den Heuvel-Eibrink, Marry M.; van der Velden, Vincent H.J.
• Is Part Of: Leukemia research. Volume 108(2021)
• Publication Date: 2021-09
• Subjects: Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2021.106652
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: Three different aberrant biological features may contribute to the pathogenesis of refractory cytopenia of childhood (RCC). I.e. the presence of minor PNH clones, TCRVβ skewing and aberrant immunophenotypes of bone marrow cells. 83.3 % of the patients with RCC displayed one or more of these aberrant features. The biological signature in RCC is heterogeneous.