Graft-versus-host disease (GVHD), rejection reaction 4/5 (2)

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GVHD
GVDH, chronic skin reaction with pigment changes. Vasconcelos L, 2013. CC BY NC 3.0

Definition

Transplanted organs (bone marrow, kidneys, heart and others) from another person can activate the immune system so that symptoms and disease occur. A similar reaction can occur after contact with other foreign substances. Graft-versus-host disease (GVHD) is a systemic disease that occurs when the graft (the tissue being transplanted such as bone marrow, kidneys or heart) is recognized as a host and attacked by the recipient's own immune cells (reference: Society G, 2014). GVHD causes symptoms that may be similar rheumatic connective tissue disorders or Vasculitis, but are often investigated and treated by immunologists. GVHD is defined as acute rejection reaction when it occurs within NUMX days, later as chronic GVHD. In order to prevent a rejection reaction, regular use of immunosuppressive drugs is essential.

Symptoms

Symptoms of Graft-versus-host disease (GVHD) from stomach and intestines, skin and fever are most common (24%). Manifestations in one organ most commonly, although combinations occur (Ferrara JL, 2009) (Vaillant AAJ, 2020). Only skin symptoms in 15%. Liver and skin affection in 7%. Liver symptoms only in 4%

The skin can be characterized by a rash on the neck, ears, shoulders, palms and soles of the feet like sunburned skin. Blisters and rashes all over the body are more serious symptoms. Some people get swollen and tight skin that can resemble systemic sclerosis/scleroderma.

Dry mouth and dry eyes (Sjögren's syndrome-like) is common and some also get ulcers in the mouth.

Gastrointestinal symptoms are seen in approx. 74%. Diarrhoea, nausea, vomiting, cramping abdominal pain are typical.

Lever attacked at approx. 44%. Apart from lethargy and jaundice (icterus) in advanced cases, the symptoms are not typical. Liver enzymes in the blood rise. ALP and conjugated bilirubin rise first (icterus).

Similar diseases / differential diagnoses

Fever of unknown cause or infections

Skin symptoms: Eczema triggered by drugs, DRESS syndrome, viral infections, radiation dermatitis (cancer treatment).

Gastrointestinal symptoms. Infections with diarrhea; clostridium, CMV or Epstein barr virus reactivation, adenovirus, rotavirus, mycobacterium avium complex, giardia, cryptosporidium. Other: thrombotic microangiopathy, bile-salt malabsorption. Nausea, vomiting, anorexia: iatrogenic secondary to chemotherapy, immunosuppression, radiation, antibiotics or opioids. (Literature: Naymagon S, 2017).

Lever. Hepatitis virus (hepatitis B, hepatitis C), drug-induced toxicity and DRESS syndrome, immunotherapy (for cancer treatment), sinusoidal obstructive syndrome (clogging of small liver veins), shock liver in case of major injuries, cancer (reference: Matsumaka KE, 2016)

Other: Drug side effect, myositis / dermatomyositis, Sjögren's syndrome, Still's disease in adulthood (Adult Stills), Systemic lupus erythematosus (SLE)

Treatment

Corticosteroids (reference: Goker H, 2001)

Experimental other treatment (reference: Murata M 2015)

Systemic treatment with corticosteroids as prednisolone is the first choice in acute GVHD (reference: Goker H, 2001). However, 35-50% of it does not have sufficient effect and needs supplementary measures (reference: Murata M 2015). The choice of second-line treatment is based on the effect of previous treatment and the risk of side effects (reference: Martin PJ, 2012). Plasmapheresis (plasma exchange) is considered in addition to drugs. These include DMARDs such as methotrexate and mycophenolate mofetil / CellCept or Biological drugs (reference: Mallard F, 2020). Most of these drugs are then used outside the approved indication, which entails the need for special information and attention.

Literature


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