Prognostic impact of Epstein-Barr virus serostatus in patients with nonmalignant hematological disorders undergoing allogeneic hematopoietic cell transplantation: the study of Infectious Diseases Working Party of the European Society for Blood and Marrow Transplantation

2020 ◽  
Vol 51 (2) ◽  
pp. 73-80 ◽  
Author(s):  
Jan Styczynski ◽  
Gloria Tridello ◽  
Lidia Gil ◽  
Per Ljungman ◽  
Malgorzata Mikulska ◽  
...  
Keyword(s):  
Epstein Barr Virus ◽  
Hematopoietic Cell Transplantation ◽  
Working Party ◽  
Hematological Disorders ◽  
Graft Versus Host ◽  
Transplant Outcomes ◽  
Barr Virus ◽  
Blood And Marrow Transplantation ◽  
Epstein Barr ◽  
Family Donor

AbstractBackgroundIn patients with acute leukemia, lymphoma and chronic malignancies, donor and/or recipient Epstein-Barr virus (EBV) seropositive status increases the risk of development of chronic graft-versus-host disease (cGVHD) after allo-hematopoietic cell transplantation (allo-HCT), while it has no influence on other transplant outcomes. No data are available on the impact of EBV serostatus on transplant outcomes in patients with nonmalignant hematological disorders.ObjectiveWe analyzed the influence of the recipient's (R) and donor's (D) EBV serostatus on transplant outcomes (overall survival (OS); relapse-free survival (RFS); relapse incidence (RI); nonrelapse mortality (NRM); acute graft-versus-host disease (aGVHD); cGVHD) in patients with nonmalignant hematological disorders undergoing allo-HCT.Patients and MethodsA total of 2,355 allo-HCTs performed between 1997 and 2016 for acquired bone marrow failure or hemoglobinopathies were included in this retrospective Registry megafile Infectious Diseases Working Party of the European Society of Blood and Marrow Transplantation (IDWP-EBMT) study.ResultsDemographics: The median age of recipient was 17.7 years (range: 0–77), and 50.8% were children. 79.0% of recipients and 75.4% of donors were EBV-seropositive. 67.8% had HCT from a matched family donor, 4.6% from a mismatched family donor, and 27.6% from an unrelated donor (UD). T-cell depletion was performed in vivo and ex vivo in 82.2% and 6.6% of patients, respectively. Conditioning regimen was myeloablative in 63.7% and reduced intensity conditioning (RIC) in 36.3% of patients. The median follow-up was 4.7 years. Transplant outcomes: EBV-seropositive recipients in comparison with EBV-seronegative recipients had lower OS (85.4% vs. 88.4%, p = 0.035) and higher NRM (10.0% vs. 6.4%, p = 0.018). No other significant differences were found for: RI, RFS, and aGVHD or cGVHD with respect to EBV pretransplant serostatus donor and/or recipient. Multivariate analysis: A trend toward higher risk of development of cGVHD (HR = 1.31; p = 0.081) and better survival (HR = 0.78; p = 0.087) in allo-HCT from EBV-seropositive donors was found. Allo-HCT in EBV-seropositive recipients had a trend toward lower risk of development of cGVHD (HR = 0.75; p = 0.065). When four subgroups (R−/D−, R−/D+, R+/D−, R+/D+ EBV serology) were analyzed, the EBV serostatus had no significant impact on OS, RFS, RI, NRM and development of aGVHD or cGVHD.ConclusionsAllo-HCT from EBV-seropositive versus EBV-seronegative donors are at 31% higher risk of cGVHD in patients with nonmalignant hematological disorders undergoing allo-HCT; however this difference is nonsignificant in multivariate analysis.

Epstein‐Barr virus associated post‐transplant lymphoproliferative disorder mimicking acute graft versus host disease

2019 ◽  
Vol 103 (5) ◽  
pp. 519-522 ◽  
Author(s):  
Shruti Prem ◽  
Zeyad Al‐Shaibani ◽  
Fotios V. Michelis ◽  
Dennis Kim ◽  
Auro Viswabandya ◽  
...  
Keyword(s):  
Graft Versus Host Disease ◽  
Lymphoproliferative Disorder ◽  
Epstein Barr Virus ◽  
Host Disease ◽  
Graft Versus Host ◽  
Post Transplant ◽  
Barr Virus ◽  
Epstein Barr ◽  
Acute Graft

scholarly journals X-linked lymphoproliferative syndromes: brothers or distant cousins?

Blood ◽  
2010 ◽  
Vol 116 (18) ◽  
pp. 3398-3408 ◽  
Author(s):  
Alexandra H. Filipovich ◽  
Kejian Zhang ◽  
Andrew L. Snow ◽  
Rebecca A. Marsh
Keyword(s):  
Epstein Barr Virus ◽  
Hematopoietic Cell Transplantation ◽  
Viral Infections ◽  
Inflammatory Responses ◽  
Lymphoproliferative Disease ◽  
Curative Therapy ◽  
Barr Virus ◽  
Clinical Consequences ◽  
Epstein Barr ◽  
Signaling Lymphocytic Activation Molecule

AbstractX-linked lymphoproliferative disease (XLP1), described in the mid-1970s and molecularly defined in 1998, and XLP2, reported in 2006, are prematurely lethal genetic immunodeficiencies that share susceptibility to overwhelming inflammatory responses to certain infectious triggers. Signaling lymphocytic activation molecule-associated protein (SAP; encoded by SH2D1A) is mutated in XLP1, and X-linked inhibitor of apoptosis (XIAP; encoded by BIRC4) is mutated in XLP2. XLP1 is a disease with multiple and variable clinical consequences, including fatal hemophagocytic lymphohistiocytosis (HLH) triggered predominantly by Epstein-Barr virus, lymphomas, antibody deficiency, and rarer consequences of immune dysregulation. To date, XLP2 has been found to cause HLH with and without exposure to Epstein-Barr virus, and HLH is commonly recurrent in these patients. For both forms of XLP, the only curative therapy at present is allogeneic hematopoietic cell transplantation. Beyond their common X-linked locus and their requirement for normal immune responses to certain viral infections, SAP and XIAP demonstrate no obvious structural or functional similarity, are not coordinately regulated with respect to their expression, and do not appear to directly interact. In this review, we describe the genetic, clinical, and immunopathologic features of these 2 disorders and discuss current diagnostic and therapeutic strategies.

scholarly journals Impact of donor and recipient Epstein-Barr Virus serostatus on outcomes of allogeneic hematopoietic cell transplantation: a systematic review and meta-analysis

2021 ◽  
Author(s):  
Michalina Kołodziejczak ◽  
◽  
Lidia Gil ◽  
Rafael de la Camara ◽  
Jan Styczyński
Keyword(s):  
Clinical Outcomes ◽  
Graft Versus Host Disease ◽  
Epstein Barr Virus ◽  
De Novo ◽  
Meta Analysis ◽  
Graft Versus Host ◽  
Hematological Diseases ◽  
Barr Virus ◽  
Epstein Barr ◽  
Meta Analyses

AbstractAllogeneic hematopoietic cell transplant (allo-HCT) is a potentially curative therapeutic strategy that showed encouraging long-term outcomes in hematological diseases. A number of factors can influence post-transplant clinical outcomes. While Epstein-Barr virus (EBV) constitutes a trigger for development of various adverse conditions, no clinical study yet has been powered to assess the effect of EBV serostatus on the clinical outcomes in allo-HCT population. To systematically summarize and analyze the impact of donor and recipient EBV serostatus on transplant outcomes in allo-HCT recipients, meta-analyses were conducted. Selected endpoints were overall survival (OS), relapse-free survival (RFS), relapse incidence (RI), non-relapse mortality (NRM), acute graft-versus-host disease (aGVHD), chronic graft-versus-host disease (cGVHD), and de novo cGVHD. Three studies with 26,650 patients, transplanted for acute leukemias, lymphomas, chronic hematological malignancies, or non-malignant hematological diseases were included in the meta-analysis. In the whole population, with a total of 53,300 donors and recipients, the rate of EBV seropositivity was 85.1%, including 86.6% and 83.6% among transplant recipients and healthy donors, respectively. Donor EBV seropositivity increased the risk of cGVHD by 17%, de novo cGVHD by 14%, and aGHVD by 5%. Recipient EBV seropositivity increased the risk of cGVHD by 12%, de novo cGVHD by 17%; increased NRM by 11%, increased RI by 11%, decreased OS by 14%, and decreased RFS by 11%. In performed meta-analyses, donor and recipient EBV seropositivity was found to have a significant impact on transplant outcomes in patients after allo-HCT.

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