Article

Concomitant EBV-related B-cell proliferation and juvenile myelomonocytic leukemia in a 2-year-old child.

Department of Pediatric Hematology and Oncology, Silesian Medical Academy, Zabrze, Poland.
Leukemia Research (impact factor: 2.92). 02/2008; 32(1):181-4. DOI:10.1016/j.leukres.2007.05.017 pp.181-4
Source: PubMed

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.

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Keywords

activating N-RAS mutation
 
concomitant EBV-related B-cell proliferation
 
EBV
 
EBV infection
 
EBV-related promotion
 
JMML pathogenesis
 
leukemic clone
 
myeloid progenitors
 
pre-existing malignant myeloid progenitors
 
predominant B-cell proliferation
 
stimulating pre-existing malignant clones