Graft-versus-host disease (GVHD) is a major complication of allogeneic hematopoietic stem cell transplantation (HSCT)caused by donor T cellsthat respond to the host polymorphic histocompatibility antigens as foreign, leading to inflammation and immune-mediated injury of host tissues. Acute GVHD (aGVHD) usually manifests within 100 days following HSCT, while chronic GVHD (cGVHD) generally occurs after 100 days and, has some features of autoimmune diseases. Clinical manifestations of aGVHD include skin rash or erythema, gastrointestinal symptoms (diarrhea, nausea, cramping, bleeding) and liver symptoms (hyperbilirubinemia and elevated serum levels of liver enzymes). Similar symptoms may occur in cGVHD, as well as nail, scalp, buccal mucosa, eye, and internal organ damage.
First line treatment for GVHD is topical and systemic corticosteroids. However, ~50% of aGVHD patients become steroid refractory, and broad spectrum immunosuppressants are commonly added ‘off label’ to a steroid regimen, leading to long-term side effects. Other options for steroid-refractory aGVHD include ruxolitib, a protein kinase inhibitor specific for JAK1 and JAK2, extracorporeal photopheresis, and various off-label treatments.
Given the choices, data, and potential risks - how do you treat steroid-refractory aGVHD ?
References
• Abedin, S. and Hamadani, M., 2020. Experimental Pharmaceuticals for Steroid-Refractory Acute Graft-versus-Host Disease. J. Exp. Pharmacology, 12, pp. 549-557. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7705269/
-
M Merrick4yrruxolitinib, ibrutinib, rituximab, ECP, among others are all options for steroid refractory GvHD -
Carolyn Steinmetz, Nurse Practitioner4yrIs there a way to risk stratify patients with steroid refractory GVHD? Do certain respond more favorably to ruxolitinib vs rituximab vs imbruvica ?
