A clinical case of a patient with scleroderma-like syndrome in chronic graft-versus host disease

Abstract

Chronic graft versus host disease is observed within the first 100 days following allogeneic haematopoietic stem cell transplantation and can affect all tissues and organs (in 80% of the cases, it affects the skin). There are some clinical correspondences between chronic graft versus host disease and certain autoimmune diseases, such as systemic scleroderma, Sjogren's syndrome, autoimmune hepatitis. We present a case of a 54-year-old man with manifested diffuse, scleroderma-like skin changes, which occurred about a year and a half after allogenic bone marrow transplantation from an unrelated donor due to a blast transformation of chronic myelomonocytic leucosis type 2. The patient was treated in a haematology clinic with corticosteroids, 10 photophoresis sessions, Ciclosporin, Tacrolimus, Mycophenolate mofetil, Imatinib. It has been assumed that this is a case of chronic graft versus host disease resistant to corticosteroids, and Methotrexate 25 mg/weekly every other month was prescribed with no significant clinical improvement. In terms of the differential diagnosis, the question remains whether or not this is a case of paraneoplastic systemic scleroderma - Autoimmune phenomena accompanying malignancy and often preceding it for months.