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

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 21.04.2021

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Synonym(s)

Cutaneous graft-versus-host disease; Graft-Host-Reaction; graft-versus-host disease; graft-versus-host reaction; GvHD; Transplant recipient response

Definition
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Complex immunological reaction with involvement of various organs, which can 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 transplant 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 side effects to various drugs, including phenobarbital, D-penicillamine, gold preparations, hepatitis B vaccine, captopril, spironolactone and allopurinol.

Classification
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From a clinical point of view (NIH consensus conference 2005), GvH reactions after their appearance in a:

  • acute graft-versus-host disease (L99.1-) (disease onset within the 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 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 after transplantation)
    • Overlap syndrome (>100 days after transplantation; signs of acute and chronic graft-versus-host disease)

to subdivide.

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

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

Literature
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  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

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 21.04.2021