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Syndromes mononucleosiques et pathologies hematologiques lies au virus d'Epstein-Barr Export

EMC - Hematologie, Vol. 2, No. 4. (December 2005), pp. 207-219.

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ebv emc hematology medicine

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ResumeLe virus d'Epstein-Barr (EBV), puissant inducteur de l'immortalisation et de la transformation des lymphocytes B, persiste a l'etat latent asymptomatique chez le sujet infecte. Ce paradoxe reflete d'une part le role du systeme immunitaire dans le controle des cellules infectees, et d'autre part les interactions entre les proteines virales et les genes cellulaires. La mononucleose infectieuse est la manifestation symptomatique de la primo-infection par l'EBV : il s'agit d'une proliferation de cellules B controlees par le systeme immunitaire. L'infection EBV chronique active, liee a la persistance de l'EBV dans des cellules T infectees, peut aboutir a une expansion clonale des lymphocytes infectes. Les mecanismes de la lymphomagenese liee a l'EBV correspondent a de profonds remaniements des cellules infectees. Dans ces cellules, des proteines virales de latence telles que la latent membrane protein (LMP-1) et l'EBV nuclear antigen (EBNA-2) induisent une veritable > de la transcription de genes cellulaires, entrainant une deregulation de l'homeostasie cellulaire avec la perte du controle de l'apoptose et du cycle cellulaire. Ces modifications de la cellule peuvent aboutir, dans des circonstances cliniques particulieres telles que les deficits immunitaires, a des proliferations lymphomateuses. Les principales pathologies lymphomateuses associees a l'EBV, outre les lymphomes des deficits immunitaires, sont le lymphome de Burkitt endemique, le lymphome hodgkinien, certains lymphomes T et NK (natural killer) comme le lymphome T de type nasal.AbstractThe Epstein-Barr virus (EBV), which preferentially infects B cells, persists in the infected subject as a latent asymptomatic infection. In adolescents, infectious mononucleosis is the symptomatic manifestation of primary EBV infection. The viral latency in the memory B-cells, the reservoir cells in peripheral blood in individuals is controlled by CD4 and CD8 positive T-cells. Immunodeficient patients are at high risk of developing EBV driven B-cell lymphomas as the consequence of the expression of oncogenic latency proteins LMP1 and EBNA2. These proteins expressed in infected B cells identify latency III or proliferation program in virus transformed B-cell, leading to lymphoid proliferation. In addition to immunodeficiency-related lymphomas, the most frequent lymphoid malignancies associated with EBV are the endemic Burkitt lymphoma, Hodgkin lymphoma and nasal type T-cell lymphoma.


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