title: Concomitant EBV-related B-cell proliferation and juvenile myelomonocytic leukemia in a 2-year-old child.
abstract: 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.
authors.affiliation: Department of Pediatric Hematology and Oncology, Silesian Medical Academy, Zabrze, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Janik-Moszant
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Barć-Czarnecka
authors.forename: Mirjam
authors.initials: M
authors.lastname: van der Burg
authors.forename: Anton W
authors.initials: AW
authors.lastname: Langerak
authors.forename: Nico G
authors.initials: NG
authors.lastname: Hartwig
authors.forename: Ann C T M
authors.initials: AC
authors.lastname: Vossen
authors.forename: Charlotte M
authors.initials: CM
authors.lastname: Niemeyer
authors.forename: Jacek
authors.initials: J
authors.lastname: Wachowiak
authors.forename: Danuta
authors.initials: D
authors.lastname: Sońta-Jakimczyk
authors.forename: Tomasz
authors.initials: T
authors.lastname: Szczepański
mesh_terms: D001402:B-Lymphocytes / Q000821:virology*; D049109:Cell Proliferation; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D006801:Humans; D054429:Leukemia, Myelomonocytic, Juvenile / Q000150:complications*; D008218:Lymphocytosis / Q000150:complications* / Q000821:virology*