Graft-versus-host disease L99.1/L99.2

Complex immunological reaction involving various organs, which may occur mainly as a result of allogeneic hematopoietic stem cell transplantation (HSCT). Rarely, but nevertheless described, is the occurrence of GvHD after transfusions of blood products, organ transplantation (liver, kidney) as well as after autologous HSCT.

In GvHR, it is mainly the donor T lymphocytes contained in the graft that react to the recipient organism. The most common symptoms of GvHR are seen in the skin (epithelial cells), liver, intestine, and eye, with the skin being the most commonly affected organ.

Graft-versus-host (GvH)-like reactions (see pseudoscleroderma below) have also been described (rarely) as adverse reactions to various drugs, including phenobarbital, D-penicillamine, gold preparations, hepatitis B vaccine, captopril, spironolactone, and allopurinol.

From a clinical perspective (NIH 2005 consensus conference), GvH reactions, once they occur, are classified as:

• acute graft-versus-host disease (L99.1-) (onset of disease within first 100 days after transplantation)
  • Persistent acute graft-versus-host disease (disease onset 100 days after transplantation).
  • Recurrent acute graft-versus-host disease (disease onset 100 days after transplantation)
  • Delayed-onset acute graft-versus-host disease
• chronic graft-versus-host disease (L99.2-) (disease onset >100 days after transplantation)
  • Classic graft versus host disease (>100 days post-transplant)
  • Overlap syndrome (>100 days after transplantation; signs of acute and chronic graft-versus-host disease)

to be subdivided.

The incidence of unrelated donors is expected to be 4-60%. With HLA incompatibility the risk of GvHD increases to 70-80%.

In > 90% of allogeneic hematopoietic stem cell transplants, the skin is involved in chronic GVHD.

1. Filipovich AH et al (2005) National Institute of Health consensus development project on criteria for clinical trials in chronic-graft-versus-host-disease. Biol Blood Marrow Transplant 11: 945-956
2. Travnik R et al (2011) Graft-versus-host-disease (GvHD) - an update. Dermatologist 62: 139-155