Graft-versus-host disease (GVHD) is a major cause of morbidity and mortality following allogeneic haematopoietic cell transplantation (HCT), which continues to limit the broader application of this therapy.
Occurs when donor T cells respond to host histoincompatible antigens on the host tissues.
Current consensus is that clinical manifestations guide whether the signs and symptoms of GVHD are acute, chronic, or an overlap syndrome.
Acute GVHD classically targets the skin, liver, and gastrointestinal tract. In contrast, chronic GVHD can involve almost any organ.
Acute GVHD prophylaxis usually comprises a calcineurin-based inhibitor such as ciclosporin or tacrolimus (that blocks T-cell activation), administered with other immunosuppressants such as low-dose methotrexate or mycophenolate.
Optimum treatment of both acute and chronic forms of GVHD is yet to be fully defined, but current practice usually involves the use of systemic corticosteroids with additional immunosuppressants as required, often as part of a clinical trial.
Supportive care and monitoring are vital components of chronic GVHD management with emphasis on infection prophylaxis, physiotherapy, nutritional status, pain control, and monitoring of drug-drug interactions and drug-related adverse effects.
Graft-versus-host disease (GVHD) is a major complication following allogeneic haematopoietic cell transplantation (HCT) and occurs when donor T cells respond to histoincompatible antigens on the host tissues.
Acute GVHD classically develops within the first 100 days of transplant or can occur beyond 100 days post-transplant with persistent, recurrent, or late-onset symptoms. The principle target organs include the skin, liver, and gastrointestinal tract.
Chronic GVHD may emerge from acute disease (progressive type), develop following a period of resolution from acute disease (quiescent or interrupted type), or occur de novo. The manifestations can be variable, and are often similar to those seen in autoimmune diseases.
Overlap syndrome is characterised by clinical features that resemble a combination of both acute and chronic GVHD.
History and exam
Key diagnostic factors
- allogeneic haematopoietic cell transplantation (HCT) recipient
- unrelated donor
- multiparous female donor
- diffuse maculopapular rash with fever
- nausea, abdominal pain, and profuse diarrhoea
Other diagnostic factors
- day +14 after HCT
- cyclophosphamide + total body irradiation (Cy/TBI) conditioning regimen
- peripheral blood stem cells as donor source
- new-onset painful mouth sores
- hyperpigmented skin lesions
- dry, gritty, and painful eyes
- dry, irritated vagina and vulva
- HLA disparity
- recipient or donor in older age group
- female donor with male recipient
- multiparous female donor
- advanced malignant condition
- high-intensity conditioning radiation regimen
- peripheral blood stem cells as source of transplant
- absent or suboptimal GVHD prophylaxis
- non-Asian or non-Hispanic ethnicity
- cytomegalovirus (CMV) seropositive
- low performance status score
- low socioeconomic status
1st investigations to order
- serum electrolytes
- liver functions tests
- urine culture
- blood culture
- stool culture
- viral polymerase chain reaction (PCR)
Investigations to consider
- CT abdomen
- Doppler ultrasound of the liver
- tissue biopsy (skin, liver, GI tract, oral lesions, or lung)
- pulmonary function tests
- high-resolution CT chest
- bronchoalveolar lavage (BAL) and culture
- barium swallow or upper GI endoscopy
- 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) scan
haematopoietic cell transplantation (HCT) recipient
acute: grade I
acute: grade II-IV
- Drug rash
- Radiation rash
- Bacterial gastroenteritis
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