How can we help patients with refractory chronic graft versus host disease – single centre experience

Chronic graft-versus-host disease (cGVHD) is a major cause of morbidity and mortality in long-term survivors of allogeneic haematopoietic stem cell transplantation (alloHSCT). Ocular involvement as well as dermal sclerosis, joint contractures and pathological changes in oral cavity are often insurmountable to treatment. These kinds of patients require complex aggressive immunosuppressive therapy. We are still waiting for drugs against cGVHD, which will be characterised by lack of infectious complications during their administration, encouraging efficacy and rare and reversible side effects. We would like to describe eight patients who developed extensive chronic graft versus host disease with eye involvement after alloHSCT. All had ocular manifestations, which were refractory to first and second line of systemic immunosuppressive therapy. All patients responded to topical cyclosporine therapy, but clinical improvement was seen from the fifth month of starting treatment. Topical cyclosporine was well tolerated. Other four patients with sclerodermoid type of skin changes, refractory to second line of systemic immunosuppressive therapy, were treated with clofazimine. Clofazimine is a drug used to treat leprosy. Because of its anti-inflammatory effects, clofazimine is also used as a second or third line of therapy for various skin conditions including: gangrenous pyoderma, lupus erythematosus and palmoplantar pustulosis and chronic graft versus host disease. All patients that received clofazimine due to dermal sclerosis, joint contractures and oral manifestations achieved partial or complete response to treatment and were able to reduce other immunosuppressive drugs. Clofazimine was generally well tolerated.

Keywords: Bone Marrow Transplantation, Immunosuppression