TY - JOUR
T1 - Outcomes for patients with EBV-positive PTLD post-allogeneic HCT after failure of rituximab-containing therapy
AU - Socié, Gérard
AU - Barba, Pere
AU - Barlev, Arie
AU - Sanz, Jaime
AU - Garcia Cadenas, Irene
AU - Chevallier, Patrice
AU - Fagioli, Franca
AU - Guzman-Becerra, Norma
AU - Kumar, Deepali
AU - Ljungman, Per
AU - Pigneux, Arnaud
AU - Sadetsky, Natalia
AU - Yáñez San Segundo, Lucrecia
AU - Shadman, Mazyar
AU - Storek, Jan
AU - Thirumalai, Dhanalakshmi
AU - Xing, Baodong
AU - Mohty, Mohamad
PY - 2023
Y1 - 2023
N2 - Epstein-Barr virus-positive (EBV +) post-transplant lymphoproliferative disease (PTLD) is an ultra-rare and aggressive condition that may occur following allogeneic hematopoietic cell transplant (HCT) due to immunosuppression. Approximately half of EBV + PTLD cases are relapsed or refractory (R/R) to initial rituximab-containing therapy. There are limited treatment options and no standard of care for patients with R/R EBV + PTLD, and little is known about their treatment history and outcomes. We performed a multinational, multicenter, retrospective chart review of patients with R/R EBV + PTLD following HCT to describe patients' demographic and disease characteristics, treatment history, and overall survival (OS) from rituximab failure. Among 81 patients who received initial treatment with rituximab as monotherapy (84.0%) or in combination with chemotherapy (16.0%), median time from HCT to PTLD diagnosis was 3.0 months and median OS was 0.7 months. Thirty-six patients received a subsequent line of treatment. The most frequent causes of death were PTLD (56.8%), graft-versus-host disease (13.5%) and treatment-related mortality (10.8%). In multivariate analysis, early PTLD onset and lack of response to initial treatment were associated with mortality. This real-world study demonstrates that the prognosis of patients with R/R EBV + PTLD following HCT remains poor, highlighting the urgent unmet medical need in this population.
AB - Epstein-Barr virus-positive (EBV +) post-transplant lymphoproliferative disease (PTLD) is an ultra-rare and aggressive condition that may occur following allogeneic hematopoietic cell transplant (HCT) due to immunosuppression. Approximately half of EBV + PTLD cases are relapsed or refractory (R/R) to initial rituximab-containing therapy. There are limited treatment options and no standard of care for patients with R/R EBV + PTLD, and little is known about their treatment history and outcomes. We performed a multinational, multicenter, retrospective chart review of patients with R/R EBV + PTLD following HCT to describe patients' demographic and disease characteristics, treatment history, and overall survival (OS) from rituximab failure. Among 81 patients who received initial treatment with rituximab as monotherapy (84.0%) or in combination with chemotherapy (16.0%), median time from HCT to PTLD diagnosis was 3.0 months and median OS was 0.7 months. Thirty-six patients received a subsequent line of treatment. The most frequent causes of death were PTLD (56.8%), graft-versus-host disease (13.5%) and treatment-related mortality (10.8%). In multivariate analysis, early PTLD onset and lack of response to initial treatment were associated with mortality. This real-world study demonstrates that the prognosis of patients with R/R EBV + PTLD following HCT remains poor, highlighting the urgent unmet medical need in this population.
KW - Haematological cancer
U2 - 10.1038/s41409-023-02127-9
DO - 10.1038/s41409-023-02127-9
M3 - Article
C2 - 37865719
SN - 0268-3369
VL - 59
SP - 52
EP - 58
JO - Bone Marrow Transplantation
JF - Bone Marrow Transplantation
ER -
