ICD-11

Graft-versus-host disease (GVHD) occurs when lymphoid cells from an immunocompetent donor are introduced into a histo-incompatible recipient incapable of rejecting them. This usually occurs as a result of haematopoietic stem cell transplantation. The main targets attacked by the donor lymphocytes are the recipient’s skin, gastrointestinal tract and liver. Acute GVHD, normally occurring within the first 100 days following transplantation, has a high mortality. The acute phase may be followed by chronic GVHD, which can also arise de novo. This usually presents as a lichenoid rash but can develop into a severe fibrosing disease affecting skin, lungs and liver.

Chronic graft-versus-host disease (GVHD) presents more than 100 days after engraftment of immunocompetent donor lymphoid cells. It has specific clinical features by which it can be distinguished from acute GVHD. It may arise de novo but frequently follows acute GVHD. Less commonly, it occurs concurrently with acute GVHD. The earlier stages of chronic GVHD are characterised by a widespread lichenoid rash, poikiloderma and involvement of nails and oral mucous membranes. If the disease remains active, progressive sclerosis of the skin and deeper tissues may result in joint contractures, fibrosis of internal organs and severe malabsorption.