Acute and chronic graft-versus-host disease treatment and management in the Eastern Mediterranean region: A Worldwide Network for Blood and Marrow Transplantation survey

Abstract

BACKGROUND: The treatment of acute and chronic graft-versus-host disease (GvHD) remains a challenge, particularly in cases of steroid-refractory GvHD. The management of GvHD varies between institutions, and little is known regarding the practices in different regions of the world. Thus, the Worldwide Network for Blood and Marrow Transplantation has developed a questionnaire to understand the current practices of GvHD management in the Eastern Mediterranean (EM) region. METHODOLOGY: The questionnaire had 46 items and was distributed electronically to transplant centers in the EM region. Responses were received between December 2022 and June 2023. The questionnaire addressed the management of acute and chronic GvHD for both newly diagnosed and refractory cases. RESULTS: The questionnaire was completed by 30 programs across 26 institutions located in 11 countries. For patients with newly diagnosed acute GvHD, most programs reported the use of systemic steroids for initial treatment, with doses selected based on the severity of the presentation: the equivalent of 1 mg/kg/day of prednisone for grade IIa and 2 mg/kg/day for grade IIb. In addition to steroids, most programs continued immunosuppressive therapy or reintroduced it if GvHD developed after its cessation. For patients who were refractory to steroids, ruxolitinib was the most frequently selected second-line treatment, chosen by 80% of the programs, followed by calcineurin inhibitors (47%), high-dose steroids (>2 mg/kg, 43%), mycophenolate mofetil (MMF, 40%), and extracorporeal photopheresis (ECP, 40%). On the other hand, for patients with newly diagnosed chronic GvHD, systemic steroids are used for the initial management of mild chronic GvHD not accessible by topical treatment and moderate to severe disease, with the most commonly used initial dose being the equivalent of 0.5 to 1 and >1 mg/kg/day of prednisone, respectively. More than two-thirds of the programs use another agent in addition to steroids in patients who develop moderate/severe chronic GvHD while off immunosuppressive therapy. For patients with steroid-refractory chronic GvHD, most programs selected multiple options in the second-line setting, with the most frequently selected options being ruxolitinib (77%), calcineurin inhibitors (68%), MMF (53%), imatinib (53%), ECP (50%), rituximab (47%), and ibrutinib (40%). CONCLUSION: Our results demonstrated that GvHD management practices in the EM region generally align with current guidelines. However, the results highlight that access to clinical trials and multidisciplinary support teams remains limited.