We present a case of a 2-year-old girl, who developed concomitant EBV-related B-cell proliferation and juvenile myelomonocytic leukemia (JMML). JMML was initially not recognized because of predominant B-cell proliferation. The activating N-RAS mutation was retrospectively already detectable at this early stage. Our findings support the hypothesis that EBV may contribute to JMML pathogenesis by stimulating pre-existing malignant clones. However, such stimulation of leukemic clone does not require the direct incorporation of the virus into myeloid progenitors. Most probably a cytokine burst resulting from EBV infection allows expansion of pre-existing malignant myeloid progenitors. Further studies are required to delineate exact mechanisms of EBV-related promotion of the JMML clone.

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Keywords Children, Epstein-Barr virus, Juvenile myelomonocytic leukemia, N-RAS mutation, Polyclonal B-lymphoproliferation
Persistent URL dx.doi.org/10.1016/j.leukres.2007.05.017, hdl.handle.net/1765/29422
Janik-Moszant, A, Barć-Czarnecka, M, van der Burg, M, Langerak, A.W, Hartwig, N.G, Vossen, A.C.Th.M, … Szczepanski, T. (2008). Concomitant EBV-related B-cell proliferation and juvenile myelomonocytic leukemia in a 2-year-old child. Leukemia Research: clinical and laboratory studies, 32(1), 181–184. doi:10.1016/j.leukres.2007.05.017