Prolonged use of immunosuppressive drugs has not been shown to prevent chronic to prevent acute graft versus host disease (GVHD).[61]Kansu E, Gooley T, Flowers ME, et al. Administration of cyclosporine for 24 months compared with 6 months for prevention of chronic graft-versus-host disease: a prospective randomized clinical trial. Blood. 2001 Dec 15;98(13):3868-70.
http://bloodjournal.hematologylibrary.org/cgi/content/full/98/13/3868
http://www.ncbi.nlm.nih.gov/pubmed/11739201?tool=bestpractice.com
There is currently no effective prophylaxis for chronic GVHD. Nonetheless, early intervention guided by a multidisciplinary approach to treatment, including appropriate immunosuppressive drugs medications and aggressive supportive care, is critical in the management of chronic GVHD.
Prophylaxis is the primary strategy to prevent acute GVHD.[62]Choi SW, Reddy P. Current and emerging strategies for the prevention of graft-versus-host disease. Nat Rev Clin Oncol. 2014 Sep;11(9):536-47.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4151470
http://www.ncbi.nlm.nih.gov/pubmed/24958183?tool=bestpractice.com
[63]Penack O, Marchetti M, Aljurf M, et al. Prophylaxis and management of graft-versus-host disease after stem-cell transplantation for haematological malignancies: updated consensus recommendations of the European Society for Blood and Marrow Transplantation. Lancet Haematol. 2024 Feb;11(2):e147-59.
http://www.ncbi.nlm.nih.gov/pubmed/38184001?tool=bestpractice.com
The most commonly used drug combination for acute GVHD prophylaxis includes a calcineurin inhibitor (cyclosporine or tacrolimus, which act to block T-cell activation) and low-dose methotrexate or mycophenolate.[64]Vogelsang GB, Arai S. Mycophenolate mofetil for the prevention and treatment of graft-versus-host disease following stem cell transplantation: preliminary findings. Bone Marrow Transplant. 2001 Jun;27(12):1255-62.
http://www.ncbi.nlm.nih.gov/pubmed/11548843?tool=bestpractice.com
[65]Storb R, Deeg HJ, Whitehead J, et al. Methotrexate and cyclosporine compared with cyclosporine alone for prophylaxis of acute graft versus host disease after marrow transplantation for leukemia. N Engl J Med. 1986 Mar 20;314(12):729-35.
http://www.ncbi.nlm.nih.gov/pubmed/3513012?tool=bestpractice.com
Evidence continues to influence the management of GVHD prophylaxis, and alternative prophylactic treatment strategies may be considered for specific patient populations.
Abatacept
A selective T-cell costimulation modulator, abatacept is approved by the Food and Drug Administration (FDA), in combination with a calcineurin inhibitor and methotrexate, for the prevention of acute GVHD in patients undergoing allogeneic hematopoietic cell transplantation (HCT) from a matched or 1 allele-mismatched unrelated donor.
In one phase 2 randomized trial, the addition of abatacept to standard prophylaxis with a calcineurin inhibitor and methotrexate numerically reduced rates of severe (grade III or IV) acute GVHD (6.8% vs. 14.8%), and significantly improved severe acute GVHD-free survival (93.2% vs. 82%), in patients with hematologic malignancies who had undergone HCT from an HLA-matched (8/8) unrelated donor.[66]Watkins B, Qayed M, McCracken C, et al. Phase II trial of costimulation blockade with abatacept for prevention of acute GVHD. J Clin Oncol. 2021 Jun 10;39(17):1865-77.
http://www.ncbi.nlm.nih.gov/pubmed/33449816?tool=bestpractice.com
Posttransplant cyclophosphamide (combined with standard prophylaxis of tacrolimus plus mycophenolate)
Increasingly favored for primary GVHD prevention based on results from large-scale, multi-site clinical trials.[63]Penack O, Marchetti M, Aljurf M, et al. Prophylaxis and management of graft-versus-host disease after stem-cell transplantation for haematological malignancies: updated consensus recommendations of the European Society for Blood and Marrow Transplantation. Lancet Haematol. 2024 Feb;11(2):e147-59.
http://www.ncbi.nlm.nih.gov/pubmed/38184001?tool=bestpractice.com
[67]Bolaños-Meade J, Hamadani M, Wu J, et al. Post-transplantation cyclophosphamide-based graft-versus-host disease prophylaxis. N Engl J Med. 2023 Jun 22;388(25):2338-2348.
https://pmc.ncbi.nlm.nih.gov/articles/PMC10575613
http://www.ncbi.nlm.nih.gov/pubmed/37342922?tool=bestpractice.com
[68]Bolaños-Meade J, Reshef R, Fraser R, et al. Three prophylaxis regimens (tacrolimus, mycophenolate mofetil, and cyclophosphamide; tacrolimus, methotrexate, and bortezomib; or tacrolimus, methotrexate, and maraviroc) versus tacrolimus and methotrexate for prevention of graft-versus-host disease with haemopoietic cell transplantation with reduced-intensity conditioning: a randomised phase 2 trial with a non-randomised contemporaneous control group (BMT CTN 1203). Lancet Haematol. 2019 Mar;6(3):e132-43.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6503965
http://www.ncbi.nlm.nih.gov/pubmed/30824040?tool=bestpractice.com
In one phase 3 trial of patients undergoing allogeneic HLA-matched HCT with reduced-intensity conditioning, GVHD-free, relapse-free survival at 1 year was significantly more common in patients randomized to cyclophosphamide-based prophylaxis (cyclophosphamide, tacrolimus, and mycophenolate) than those assigned to standard prophylaxis (52.7% vs. 34.9%).[67]Bolaños-Meade J, Hamadani M, Wu J, et al. Post-transplantation cyclophosphamide-based graft-versus-host disease prophylaxis. N Engl J Med. 2023 Jun 22;388(25):2338-2348.
https://pmc.ncbi.nlm.nih.gov/articles/PMC10575613
http://www.ncbi.nlm.nih.gov/pubmed/37342922?tool=bestpractice.com
Posttransplant cyclophosphamide-based prophylaxis is commonly used in patients who have undergone allogeneic HCT from an HLA-haploidentical (i.e., half-matched) donor or unrelated donor.[69]Luznik L, O'Donnell PV, Symons HJ, et al. HLA-haploidentical bone marrow transplantation for hematologic malignancies using nonmyeloablative conditioning and high-dose, posttransplantation cyclophosphamide. Biol Blood Marrow Transplant. 2008 Jun;14(6):641-50.
https://pmc.ncbi.nlm.nih.gov/articles/PMC2633246
http://www.ncbi.nlm.nih.gov/pubmed/18489989?tool=bestpractice.com
[70]Gooptu M, Antin JH. GVHD Prophylaxis 2020. Front Immunol. 2021;12:605726.
https://pmc.ncbi.nlm.nih.gov/articles/PMC8059368
http://www.ncbi.nlm.nih.gov/pubmed/33897681?tool=bestpractice.com
Rabbit anti-thymocyte globulin
A polyclonal immunoglobulin G, rabbit anti-thymocyte globulin reduces the cumulative incidence of both acute and chronic GVHD in patients undergoing HCT from unrelated donors. In one randomized phase 3 trial, the addition of rabbit anti-thymocyte globulin to standard prophylaxis (cyclosporine or tacrolimus plus methotrexate or mycophenolate) reduced acute GVHD incidence at 30 and 100 days compared with standard prophylaxis (30 days: 22% vs. 37%; 100 days: 50% vs. 65%).[71]Walker I, Panzarella T, Couban S, et al. Pretreatment with anti-thymocyte globulin versus no anti-thymocyte globulin in patients with haematological malignancies undergoing haemopoietic cell transplantation from unrelated donors: a randomised, controlled, open-label, phase 3, multicentre trial. Lancet Oncol. 2016 Feb;17(2):164-73.
http://www.ncbi.nlm.nih.gov/pubmed/26723083?tool=bestpractice.com
At 24 months, this regimen reduced incidence of chronic GVHD (26.3% vs. 41.3%), and lead to improved survival (70.6% vs. 53.3%) and reduced use of immunosuppressive therapy.[72]Walker I, Panzarella T, Couban S, et al. Addition of anti-thymocyte globulin to standard graft-versus-host disease prophylaxis versus standard treatment alone in patients with haematological malignancies undergoing transplantation from unrelated donors: final analysis of a randomised, open-label, multicentre, phase 3 trial. Lancet Haematol. 2020 Feb;7(2):e100-11.
http://www.ncbi.nlm.nih.gov/pubmed/31958417?tool=bestpractice.com
Rabbit anti-thymocyte globulin effectively reduces GVHD incidence after HLA-matched sibling donor HCT.[73]Chang YJ, Wu DP, Lai YR, et al. Antithymocyte globulin for matched sibling donor transplantation in patients with hematologic malignancies: a multicenter, open-label, randomized controlled study. J Clin Oncol. 2020 Oct 10;38(29):3367-76.
https://ascopubs.org/doi/10.1200/JCO.20.00150?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
http://www.ncbi.nlm.nih.gov/pubmed/32650683?tool=bestpractice.com
Sirolimus combined with standard prophylaxis (cyclosporine plus mycophenolate)
Lowers the incidence of grade II to IV acute GVHD (at day 100) compared with cyclosporine plus mycophenolate alone in patients who have undergone allogeneic HCT from an HLA-matched unrelated donor with non-myeloablative conditioning.[74]Sandmaier BM, Kornblit B, Storer BE, et al. Addition of sirolimus to standard cyclosporine plus mycophenolate mofetil-based graft-versus-host disease prophylaxis for patients after unrelated non-myeloablative haemopoietic stem cell transplantation: a multicentre, randomised, phase 3 trial. Lancet Haematol. 2019 Aug;6(8):e409-18.
https://pmc.ncbi.nlm.nih.gov/articles/PMC6686903
http://www.ncbi.nlm.nih.gov/pubmed/31248843?tool=bestpractice.com
Tacrolimus plus sirolimus
Suggested as an alternative regimen to standard GVHD prophylaxis.[75]Antin JH, Kim HT, Cutler C, et al. Sirolimus, tacrolimus, and low-dose methotrexate for graft-versus-host disease prophylaxis in mismatched related donor or unrelated donor transplantation. Blood. 2003 Sep 1;102(5):1601-5.
http://bloodjournal.hematologylibrary.org/cgi/content/full/102/5/1601
http://www.ncbi.nlm.nih.gov/pubmed/12730113?tool=bestpractice.com
[76]Cutler C, Li S, Ho VT, et al. Extended follow-up of methotrexate-free immunosuppression using sirolimus and tacrolimus in related and unrelated donor peripheral blood stem cell transplantation. Blood. 2007 Apr 1;109(7):3108-14.
http://bloodjournal.hematologylibrary.org/cgi/content/full/109/7/3108
http://www.ncbi.nlm.nih.gov/pubmed/17138818?tool=bestpractice.com
[77]Törlén J, Ringdén O, Garming-Legert K, et al. A prospective randomized trial comparing cyclosporine/methotrexate and tacrolimus/sirolimus as graft-versus-host disease prophylaxis after allogeneic hematopoietic stem cell transplantation. Haematologica. 2016 Nov;101(11):1417-25.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5394879
http://www.ncbi.nlm.nih.gov/pubmed/27662016?tool=bestpractice.com
[78]Cutler C, Logan B, Nakamura R, et al. Tacrolimus/sirolimus vs tacrolimus/methotrexate as GVHD prophylaxis after matched, related donor allogeneic HCT. Blood. 2014 Aug 21;124(8):1372-7.
http://www.bloodjournal.org/content/124/8/1372.long?sso-checked=true
http://www.ncbi.nlm.nih.gov/pubmed/24982504?tool=bestpractice.com
Incidence of acute GVHD (by day +114) is similar between tacrolimus plus sirolimus and tacrolimus plus methotrexate in patients who have undergone allogeneic HCT from a matched related donor.[78]Cutler C, Logan B, Nakamura R, et al. Tacrolimus/sirolimus vs tacrolimus/methotrexate as GVHD prophylaxis after matched, related donor allogeneic HCT. Blood. 2014 Aug 21;124(8):1372-7.
http://www.bloodjournal.org/content/124/8/1372.long?sso-checked=true
http://www.ncbi.nlm.nih.gov/pubmed/24982504?tool=bestpractice.com