Chronic graft versus host disease

Other Names: Chronic GVHD Chronic graft versus host disease (GVHD) is a complication that can occur after a stem cell or bone marrow transplant in which the newly transplanted donor cells attack the transplant recipient's body. Graft-versus-host disease can cause damage to the host’s tissues and organs, especially the skin, liver, intestines, eyes, mouth, hair, nails, joints, muscles, lungs, kidneys, and genitals.

GvHD pathology

Symptoms

The signs and symptoms may be severe and life threatening. Graft-versus-host disease can occur within the first few months after transplant (acute) or much later (chronic). Symptoms may include skin rash, mouth sores, dry eyes, liver inflammation, development of scar tissue in the skin and joints, and damage to the lungs.

cause

The exact cause of chronic GVHD is unknown. It likely results from a complex immune-mediated interaction between the donor and recipient cells. Three criteria, known as the Billingham criteria, must be met in order for GvHD to occur.

• An immuno-competent graft is administered, with viable and functional immune cells.
• The recipient is immunologically different from the donor – histo-incompatible.
• The recipient is immunocompromised and therefore cannot destroy or inactivate the transplanted cells.

Treatment

Intravenously administered glucocorticoids, such as prednisone, are the standard of care in acute GvHD Chronic GVHD is treated with prednisone or other similar anti-inflammatory or immunosuppressive medications. The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Ibrutinib(Brand name: Imbruvica)Treatment of adult patients with chronic graft versus host disease (cGVHD).

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NIH genetic and rare disease info

• NIH info on Chronic graft versus host disease

Chronic graft versus host disease is a rare disease.