“…Two entities have classically been considered, systemic DLBCL and DLBCL with exclusive involvement of the central nervous system ( Figure 1 ) [ 30 ]. Although the WHO classification reserves the term primary DLBCL of the central nervous system (PCNSL) for immunocompetent patients, PLWH typically have DLBCL with exclusive involvement of the CNS, which is considered an AIDS-defining condition, thus the term PCNSL is commonly found in the literature in the HIV setting [ 31 ].…”
Section: Implications Of Epstein–barr Virus In the Different Hiv-related Lymphoma Typesmentioning
confidence: 99%
“…PCNSL is an infrequent lymphoma affecting 15 per 100,000 persons-year in PLWH [ 32 ]. HIV is considered a risk factor in this lymphoma, but its incidence has dramatically decreased in the cART era [ 31 , 32 , 41 , 42 ]. Nearly all cases (80–100%) of HIV-related PCNSL are associated with EBV [ 27 , 43 , 44 , 45 , 46 ].…”
Section: Implications Of Epstein–barr Virus In the Different Hiv-related Lymphoma Typesmentioning
confidence: 99%
“…PCNSL usually occurs in patients with advanced immunosuppressed status and low CD4+ lymphocyte counts (<50 cells/µL), and frequently with an AIDS-defining illness before lymphoma diagnosis [ 31 , 45 , 64 , 65 , 66 ]. In this regard, low CD4+ lymphocyte counts and high HIV load are associated with a worse outcome in patients with PCNSL [ 31 , 42 ].…”
Section: Implications Of Epstein–barr Virus In the Different Hiv-related Lymphoma Typesmentioning
confidence: 99%
“…PCNSL usually occurs in patients with advanced immunosuppressed status and low CD4+ lymphocyte counts (<50 cells/µL), and frequently with an AIDS-defining illness before lymphoma diagnosis [ 31 , 45 , 64 , 65 , 66 ]. In this regard, low CD4+ lymphocyte counts and high HIV load are associated with a worse outcome in patients with PCNSL [ 31 , 42 ]. Given that EBV coinfection is detected in nearly all HIV-PCNSL patients, the detection of EBV-DNA in cerebrospinal fluid (CSF) is a quick diagnostic tool for HIV-related PCNSL diagnosis, having a high sensitivity and specificity [ 45 , 67 ].…”
Section: Implications Of Epstein–barr Virus In the Different Hiv-related Lymphoma Typesmentioning
The incidence of lymphomas is increased in people living with HIV (PLWH). Aggressive B-cell non-Hodgkin lymphomas (NHLs) are the most common and are considered an AIDS-defining cancer (ADC). Although Hodgkin lymphoma (HL) is not considered an ADC, its incidence is also increased in PLWH. Among all HIV-related lymphomas (HRL), the prevalence of Epstein–Barr virus (EBV) is high. It has been shown that EBV is involved in different lymphomagenic mechanisms mediated by some of its proteins, contributing to the development of different lymphoma subtypes. Additionally, cooperation between both HIV and EBV can lead to the proliferation of aberrant B-cells, thereby being an additional lymphomagenic mechanism in EBV-associated HRL. Despite the close relationship between EBV and HRL, the impact of EBV on clinical aspects has not been extensively studied. These lymphomas are treated with the same therapeutic regimens as the general population in combination with cART. Nevertheless, new therapeutic strategies targeting EBV are promising for these lymphomas. In this article, the different types of HRL are extensively reviewed, focusing on the influence of EBV on the epidemiology, pathogenesis, clinical presentation, and pathological characteristics of each lymphoma subtype. Moreover, novel therapies targeting EBV and future strategies to treat HRL harboring EBV are discussed.
“…Two entities have classically been considered, systemic DLBCL and DLBCL with exclusive involvement of the central nervous system ( Figure 1 ) [ 30 ]. Although the WHO classification reserves the term primary DLBCL of the central nervous system (PCNSL) for immunocompetent patients, PLWH typically have DLBCL with exclusive involvement of the CNS, which is considered an AIDS-defining condition, thus the term PCNSL is commonly found in the literature in the HIV setting [ 31 ].…”
Section: Implications Of Epstein–barr Virus In the Different Hiv-related Lymphoma Typesmentioning
confidence: 99%
“…PCNSL is an infrequent lymphoma affecting 15 per 100,000 persons-year in PLWH [ 32 ]. HIV is considered a risk factor in this lymphoma, but its incidence has dramatically decreased in the cART era [ 31 , 32 , 41 , 42 ]. Nearly all cases (80–100%) of HIV-related PCNSL are associated with EBV [ 27 , 43 , 44 , 45 , 46 ].…”
Section: Implications Of Epstein–barr Virus In the Different Hiv-related Lymphoma Typesmentioning
confidence: 99%
“…PCNSL usually occurs in patients with advanced immunosuppressed status and low CD4+ lymphocyte counts (<50 cells/µL), and frequently with an AIDS-defining illness before lymphoma diagnosis [ 31 , 45 , 64 , 65 , 66 ]. In this regard, low CD4+ lymphocyte counts and high HIV load are associated with a worse outcome in patients with PCNSL [ 31 , 42 ].…”
Section: Implications Of Epstein–barr Virus In the Different Hiv-related Lymphoma Typesmentioning
confidence: 99%
“…PCNSL usually occurs in patients with advanced immunosuppressed status and low CD4+ lymphocyte counts (<50 cells/µL), and frequently with an AIDS-defining illness before lymphoma diagnosis [ 31 , 45 , 64 , 65 , 66 ]. In this regard, low CD4+ lymphocyte counts and high HIV load are associated with a worse outcome in patients with PCNSL [ 31 , 42 ]. Given that EBV coinfection is detected in nearly all HIV-PCNSL patients, the detection of EBV-DNA in cerebrospinal fluid (CSF) is a quick diagnostic tool for HIV-related PCNSL diagnosis, having a high sensitivity and specificity [ 45 , 67 ].…”
Section: Implications Of Epstein–barr Virus In the Different Hiv-related Lymphoma Typesmentioning
The incidence of lymphomas is increased in people living with HIV (PLWH). Aggressive B-cell non-Hodgkin lymphomas (NHLs) are the most common and are considered an AIDS-defining cancer (ADC). Although Hodgkin lymphoma (HL) is not considered an ADC, its incidence is also increased in PLWH. Among all HIV-related lymphomas (HRL), the prevalence of Epstein–Barr virus (EBV) is high. It has been shown that EBV is involved in different lymphomagenic mechanisms mediated by some of its proteins, contributing to the development of different lymphoma subtypes. Additionally, cooperation between both HIV and EBV can lead to the proliferation of aberrant B-cells, thereby being an additional lymphomagenic mechanism in EBV-associated HRL. Despite the close relationship between EBV and HRL, the impact of EBV on clinical aspects has not been extensively studied. These lymphomas are treated with the same therapeutic regimens as the general population in combination with cART. Nevertheless, new therapeutic strategies targeting EBV are promising for these lymphomas. In this article, the different types of HRL are extensively reviewed, focusing on the influence of EBV on the epidemiology, pathogenesis, clinical presentation, and pathological characteristics of each lymphoma subtype. Moreover, novel therapies targeting EBV and future strategies to treat HRL harboring EBV are discussed.
“…The former is estimated to account for up to 1%-2% of NHLs and approximately 3%-5% of all primary brain tumors. The latter is found mainly in patients suffering from human immunodeficiency virus (HIV) infection, leading to the sharp increase in PCNSLs in immunosuppressed patients in recent decades (1,(5)(6)(7).…”
Primary central nervous system lymphoma (PCNSL) is a rare subtype of extra-nodal lymphoma. The high relapse rate of PCNSL remains a major challenge to the hematologists, even though patients exhibit high sensitivity to the methotrexate-based chemotherapeutic regimens. Recently, the advent of Bruton’s tyrosine kinase inhibitor (BTKi) and CAR T treatment has made more treatment options available to a proportion of patients. However, whether BTKi monotherapy should be given alone or in combination with conventional chemotherapy is still a clinical question. The status of CAR T therapy for PCNSLs also needs to be elucidated. In this review, we summarized the latest progress on the epidemiology, pathology, clinical manifestation, diagnosis, and treatment options for PCNSLs.
To explore prognostic factors and outcomes of primary central nervous system lymphoma (PCNSL) of diffuse large B-cell lymphoma (DLBCL) in Taiwan, 124 PCNSL-DLBCL patients (from 1995 to 2021) were retrospectively analyzed. Mainly, two treatment modalities including sandwich chemoradiotherapy and modified MATRix regimen were employed in these patients. Overall survival (OS) was determined by log-rank test and time-dependent Cox analysis. Median OS of all patients was 27.1 months. 47 (37.9%) patients who underwent sandwich chemoradiotherapy had a complete remission (CR) rate of 87.2%, median OS of 53.9 months, and progression free survival (PFS) of 42.9 months. 11 (8.9%) patients who underwent modified MATRix regimen had CR rate of 72.7%, median OS of 18.9, and PFS of 11.2 months. There are no significant OS differences between treatment groups or addition of Rituximab. Patients treated with the modified MATRix regimen experienced a higher early mortality rate followed by a survival plateau. IELSG low-risk group had significantly improved OS and PFS than IELSG intermediate- or high-risk group. In multivariant analysis, age > 60 years old and bilateral cerebral lesions are associated with significantly inferior OS. Sandwich chemoradiotherapy demonstrated better early survival and reduced treatment-related toxicity for PCNSL patients compared to the modified MATRix regimen. However, the long-term follow-up revealed a higher rate of treatment failure events in the sandwich chemoradiotherapy group. IELSG and MSKCC scores served as reliable risk assessment models. Incorporating bilateral cerebral lesions as a risk factor further improved risk evaluation.
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