Abstract:The aim of the present study was to confirm the histopathological features of aggressive B-cell lymphoma in Papua New Guinea (PNG)-an EBV endemic region. The immunophenotypic features and expression of EBV-encoded proteins and RNA in B-cell lymphomas were analyzed in 21 PNG children, and compared to the corresponding features of 17 Japanese children with Burkitt lymphoma (BL). Histological diagnosis of the lymphomas from the PNG children was BL in nine patients; atypical Burkitt/Burkitt-like variant of BL (BLL… Show more
“…Sporadic cases in nonimmunocompromised hosts were described in some Asian, African, and Latin America countries, where EBV infection is present in 70% to 80% of children by the age of 2 years. In Malaysia, 2 of 5 cases of DLBCL were EBV+ [8]; in Papua New Guinea, 1 of 4 cases was EBV+ [9]. Recently, it was reported that 10 of 25 cases of pediatric DLBCL occurring in Argentina were EBV+ [10].…”
“…Sporadic cases in nonimmunocompromised hosts were described in some Asian, African, and Latin America countries, where EBV infection is present in 70% to 80% of children by the age of 2 years. In Malaysia, 2 of 5 cases of DLBCL were EBV+ [8]; in Papua New Guinea, 1 of 4 cases was EBV+ [9]. Recently, it was reported that 10 of 25 cases of pediatric DLBCL occurring in Argentina were EBV+ [10].…”
“…Regarding EBV-host interactions, four different forms of virus latency have been recognized based on the rate of expression of EBV-encoded proteins and RNA. 35 Type 1, which is observed in Burkitt lymphoma, shows exclusive expression of EBV-encoded nuclear antigen 1 (small non-polyadenylated RNA (EBER) molecules and EBVencoded nuclear antigen 1 (EBNA-1)); type II, which is observed in Hodgkin's lymphoma, shows additional expression of LMP; type III, which is observed in lymphoproliferative disease (LPD) in immunocompromised patients, shows expression of all six EBNAs, all three LMPs and the two EBERs; type IV latency is less strictly defined and pertains to infectious mononucleosis and post-transplantation LPD. In the six cases of primary CNS EBV-positive diffuse large B-cell lymphomas of the elderly that we reported, we found that all cases examined expressed EBV EBNA1, EBNA2 and LMP1.…”
Section: Diffuse Large B-cell Lymphomas and Burkitt Lymphomasmentioning
Although primary diffuse large B-cell lymphomas of the CNS are designated as primary CNS lymphomas according to the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue in 2008, a variety of other lymphomas (Burkitt lymphomas, EBV-positive diffuse large B-cell lymphoma of the elderly) and related diseases (lymphomatoid granulomatosis) that are also found in the CNS have been spotlighted in recent years. The histopathology of primary CNS Burkitt lymphomas mimics that of primary diffuse large B-cell lymphomas of the CNS after steroid administration. Therefore, for correct diagnosis of the involved lymphoma, comprehensive fluorescent in situ hybridization analysis for c-MYC and BCL2 is recommended in all primary CNS lymphoma cases with aggressive clinical course, multifocal involvement of the CNS, and a high proliferation index. The pathological characteristics of primary CNS EBV-positive diffuse large B-cell lymphoma of the elderly have similarities with those of the latency phenotype III, EBV lymphoproliferative disorders that arise in the setting of immunodeficiency. These age-related lymphomas usually occur in elderly immunocompetent patients, and the incidence of this disease was estimated to range from 4.0% to 13.6% of all primary CNS lymphomas. Shorter overall survival has been reported for patients with this disease. Lymphomatoid granulomatosis (LYG) is a systemic, EBV-driven, angiocentric and angiodestructive lymphoproliferative disorder. Primary LYG that shows distinct clinicopathological features compared with systemic LYG was recently reported. Finally, this review focuses on the relationship between primary CNS lymphomas and demyelinating diseases, and the concomitant use of intraoperative cytology and frozen sections that are helpful in rapid intraoperative diagnosis.
“…In Israel, only 34% of BL cases have been shown to be EBER positive . In India and in Papua New Guinea, EBER was detected in 83‐91% of BL cases . Despite the similar climatic conditions and holoendemic malaria transmission in Malaysia, only 27% of BL cases harbored EBV; although nonsignificant, ethnic differences were observed, which suggest an ethnic predisposition .…”
Section: Discussion and Literature Reviewmentioning
Background
Epstein‐Barr virus (EBV) is linked to a variety of malignancies; most endemic Burkitt lymphoma (BL) harbor EBV, whereas only a subset of the cases of sporadic BL is EBV positive.
Procedure
We retrospectively determined the herpesvirus seroprevalence at the time of diagnosis in pediatric non‐Hodgkin lymphoma (NHL) patients enrolled in NHL‐BFM (Berlin‐Frankfurt‐Muenster) studies. We accessed the seroepidemiological data from 1147 patients that became available during 1990‐2007. We included the records from patients 6 months to 18 years of age with BL, T‐cell lymphoblastic lymphoma (T‐LBL), lymphoblastic precursor B‐cell lymphoma (pB‐LBL), diffuse large B‐cell lymphoma (DLBCL), or anaplastic large cell lymphoma (ALCL).
Results
EBV seropositivity was significantly more frequent in patients with BL than in those with T‐LBL. EBV was more prevalent in patients younger than 6 years of age and in patients with BL than in those with non‐BL or T‐LBL. Event‐free survival was significantly lower in varicella‐zoster–seronegative patients, but there was no indication of an association to complications due to varicella zoster infection. We found no associations between herpes simplex virus, varicella zoster virus, or human cytomegalovirus seroprevalence and the pediatric Central European NHL cases.
Conclusion
Early EBV exposure may increase the risk of BL in Central Europe. A higher involvement of EBV in European BL than originally reported appears at least probable. Our data support the thesis that the distinction between endemic and sporadic BL is artificial and should be replaced by the differentiation between EBV‐positive and EBV‐negative BL.
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