EbV-positive diffuse large cell b-cell lymphoma C83.7

Epstein-Barr virus-positive, diffuse large B-cell lymphoma (EBV-positive DLBCL) is usually an aggressive (highly malignant) non-Hodgkin's lymphoma. DLBCL is the most common subtype of malignant B-cell lymphoma. The EBV-positive variant of diffuse large B-cell lymphoma was detected in 6.9 % of cases in a larger study (n=239) of newly diagnosed patients using in situ hybridization.

The incidence of EBV-positive DLBCL in Asia and Latin America is 8 to 10 % (Poston JN et al).

Epstein-Barr virus (EBV) positivity in diffuse large B-cell lymphoma (DLBCL) triggers a crucial oncogenic mechanism to activate intracellular signaling through LMP1 (latent membrane protein 1). LMP1, an Epstein-Bar virus specific protein, acts as a functional CD40 homologue to prevent apoptosis of infected B lymphocytes and promote their proliferation. The protein functions as a constitutively active tumor necrosis factor receptor that induces activation of several signaling pathways, including those of the NF-kappa B family. LMP1 signaling leads to the upregulation of anti-apoptotic proteins and provides growth signals in latently infected cells.

Therapeutically, successes are described by rituximab , with a similar success as in EBV-negative lymphomas. The median survival time and progression-free survival time of this group was 8.7 and 6.8 months, respectively, after retuximab therapy of patients with EBV-positive DLBL (Sato A et al. 2014).

It is striking that the African form is geographically limited to areas where malaria is also endemic (Mawson AR et al. 2017). The fact that the incidence of Burkitt's lymphoma is also significantly lower in those areas of Africa where malaria is repressed shows a close pathogenetic link between the two lymphoma types. The connections have not yet been clarified. However, it has been shown that a malaria attack is associated with a significantly reduced EBV-specific cytotoxicity. Therefore, it seems to be possible that EBV-transformed B-lymphocytes can prolong proliferation.

1. Poston JN et al (2018) The Diving Bell and the Butterfly Revisited: A Fatal Case of Locked-in Syndrome in a Man With Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified. Clin Med Insights Blood Disord11:1179545X18762799.

2. Mawson AR et al (2017) Malaria, Epstein-Barr virus infection and thepathogenesis of
   Burkitt's lymphoma. Int J Cancer 141:1849-1855.

3. Sato A (2014) Clinical outcome of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly in the rituximab era. Cancer Sci 105:1170-1175.

4. Yoon SE et al (2020) A phase II study of ibrutinib in combination with rituximab-cyclophosphamide-doxorubicin hydrochloride-vincristine sulfate-prednisone therapy in Epstein-Barr virus-positive, diffuse large B cell lymphoma (54179060LYM2003: IVORY study): results of the final analysis. Ann Hematol 99:1283-1291.