Graft-versus-host disease acute L99.1-

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Acute Graft-versus-Host Disease; Acute GvHD

Definition
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Disease caused by the transmission of allogeneic immunocompetent donor T lymphocytes within the first 100 days after transplantation with abdominal pain, diarrhoea, liver dysfunction and skin changes. Acute GvHD triit mainly after myeloablative conditioning with total body irradiation with 12 Gy.

Etiopathogenesis
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The transfer of allogeneic immunocompetent lymphocytes, e.g. in bone marrow transplantation, leads to a specific immunological reaction of the lymphocytes against the body tissue of the recipient. Target cells are in particular the skin, liver and gastrointestinal tract.

Manifestation
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Occurs at any age, depending on the time of pre-treatment.

Localization
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Typically, the face, palms and soles of the feet are affected, with the chronically light-exposed areas showing more severe symptoms.

Clinical features
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Itching, pressure pain and erythema palmoplantar and retroauricular. Within 24 hours a polymorphic, mostly maculopapular, morbilliform or scarlatiniform exanthema develops; however, haemorrhagic aspects are also found. As a maximum form a bullous GvHD can develop under the picture of a toxic epidermal necrolysis (TEN) of skin and mucosa. Mucosal involvement in the context of acute GvHD manifests itself in the form of a mostly painful mucositis, with unspecific enanthema, but also lichenoid, reticular ( lichen planus-like) drawings. These are usually associated with xerostomia, to be interpreted as an "overlap syndrome", the simultaneous occurrence of acute and chronic GvHD.

External therapy
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According to studies the treatment of the pruritus by means of topical application of 0,1% tacrolimus ointment is examined. 72% of the patients reported a significant reduction of itching.

Radiation therapy
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Dermatologically relevant is the early application of PUVA therapy, which shows good results in acute GVHR and can also reduce the rate of patients with transition to chronic GVRH. Initial dose 0.5-0.25 J/cm2 at 0.6 mg/kg bw/day of methoxsalene (e.g. meladinine), dose increase up to 8 J/cm2 over several sessions. Maintenance therapy 1-2 times/week for 1-2 years.

Internal therapy
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From grade II on, systemic immunosuppression with systemic glucocorticoids such as prednisolone (e.g. Decortin H) 100 mg/day in combination with Ciclosporin A (sandimmune) 5 mg/kg bw/day. In case of non-response, antithymocyte globin i.v. can be used additionally. Therapy trials with monoclonal antibodies, e.g. against the alpha/beta T-cell receptor, are possible. The survival rate is extremely low with grade IV GVHR.

Prophylaxis
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The prophylaxis of an acute Graft-vs-Host disease is performed with orally administered Ciclosporin A or optionally Tacrolimus in combination with either methotrexate or mycophenolate mofetil. Against the background of an allogeneic stem cell transplantation or in case of family donation with an increased risk for a graft-vs host reaction, the therapy scheme can be supplemented with anti-T-cell immunoglobulin if necessary. Effective levels of Ciclosporin A should already be reached at the time of transplantation and prophylaxis should be started before then. The optional administration of tacrolimus is especially suitable if hepatotoxic side effects are observed under Ciclosporin therapy.

Tables
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Staging of acute Graft-versus-Host Disease (according to Glucksberg et al.)

Graduation

Percentage of body surface area (%)

Skin Appearances

Histology

Grade I

< 25%

Maculopapular exanthema

Vacuolisation of basal cells, lymphocytic inflammatory infiltrate in the upper dermis or epidermis

Grade II

> 25-50%

Maculopapular exanthema

Dyskeratosis of individual keratinocytes, exocytosis of lymphocytes into the immediate vicinity of necrotic keratinocytes in the epidermis (satellite phenomenon)

Grade III

> 50%

Erythrodermy

Beginning formation of clefts in the basement membrane zone, partial necrosis of the epidermis

Grade IV

Blistering and toxic epidermal necrolysis

Complete removal of the necrotic epidermis

Literature
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  1. Eppinger T et al (1990) 8-Methoxypsoralen and ultraviolet a therapy of cutaneous manifestations of graft-versus-host disease. Transpl 50: 807-811
  2. Karrer S (2003) Cutaneous graft-versus-host disease. dermatologist 54: 465-480
  3. Reinauer S et al (1993) Photochemotherapy (PUVA) of acute graft-versus-host disease. dermatologist 44: 708-712
  4. Stander S, Luger TA (2003) Antipruritic effects of pimecrolimus and tacrolimus. dermatologist 54: 413-417
  5. Volc-Place B (1992) Graft-versus-host reaction (GvHD). dermatologist 43: 669-675

Disclaimer

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

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Last updated on: 29.10.2020