ABSTRACT
Chronic graft-versus-host disease (GVHD) is the most common late complication of allogeneic bone marrow transplantation (BMT). The sclerodermatous form of the disease is often refractory to standard treatment modalities. Based on reports of response to etretinate, a synthetic retinoid, among patients with scleroderma, we have added etretinate to the treatment regimen of 32 patients with refractory sclerodermatous chronic GVHD. This case series is comprised mainly of patients who had chronic GVHD of long duration (median of 30 months before the initiation of etretinate). Most had failed to respond to three or more agents before etretinate treatment was started. Clinical response was assessed after 3 months of therapy. Five patients did not complete a 3-month trial. Among the 27 patients evaluable for response, 20 showed improvement including softening of the skin, flattening of cutaneous lesions, increased range of motion, and improved performance status. Four showed no response after 3 months of therapy and 3 had progression of their sclerosis. Overall, etretinate has been fairly well tolerated in our patients, with skin breakdown and/or ulceration leading to its discontinuation in 6 patients. We believe the results in our patients are encouraging and suggest that further evaluation of etretinate in the treatment of sclerodermatous chronic GVHD is warranted.
Subject(s)
Etretinate/therapeutic use , Graft vs Host Disease/drug therapy , Scleroderma, Systemic/drug therapy , Adolescent , Adult , Bone Marrow Transplantation/adverse effects , Child , Child, Preschool , Chronic Disease , Etretinate/adverse effects , Female , Graft vs Host Disease/etiology , Graft vs Host Disease/immunology , Humans , Male , Middle Aged , Scleroderma, Systemic/etiology , Scleroderma, Systemic/immunology , Treatment OutcomeABSTRACT
BACKGROUND: Graft-vs-host disease (GVHD) represents one of the major complications of allogeneic bone marrow transplantation (BMT) but is less common in autologous BMT. Following autologous BMT, chronic GVHD has been reported in only four patients, all of whom had a self-limited sclerodermoid form. Lichenoid chronic GVHD has not been previously reported in an autologous BMT patient. OBSERVATIONS: Mucosal and cutaneous lichenoid lesions and histologic findings compatible with chronic lichenoid GVHD developed in a patient 35 days after autologous BMT was performed. The onset of clinical lesions at 35 days after BMT is not incongruent with the diagnosis of chronic lichenoid GVHD (rather than a graft-vs-host reaction) and may have been augmented by cyclosporin A in a manner similar to animal model experiments. CONCLUSION: All forms of GVHD can and do occur following autologous BMT.
Subject(s)
Bone Marrow Transplantation/adverse effects , Graft vs Host Disease/etiology , Lichenoid Eruptions/etiology , Adult , Chronic Disease , Graft vs Host Disease/pathology , Humans , Lichenoid Eruptions/pathology , MaleABSTRACT
BACKGROUND: Chronic graft-vs-host disease (GVHD) is a late complication of allogeneic bone marrow transplantation and is associated with high morbidity and mortality. While the pathogenesis of chronic GVHD is not fully understood, several observations and studies suggest that viral infections may play a role. We describe two patients who developed linear lichenoid chronic GVHD. The dermatomal distribution of their lesions suggests an association with herpes zoster virus infection. OBSERVATIONS: Two allogeneic bone marrow transplantation patients developed violaceous papules in a dermatomal distribution. Histologic examination of these lesions revealed dyskeratosis, vacuolar changes in the basal layer, and a mild perivascular and interstitial infiltrate, diagnostic of lichenoid chronic GVHD. CONCLUSIONS: The linear distribution of our patients' lichenoid chronic GVHD is unique and may represent an association with herpes zoster virus infection, providing further support for a role for viral infections in the pathogenesis of chronic GVHD.
