acute graft versus host disease
acute graft versus host disease
A distinctive syndrome of dermatitis, hepatitis, and enteritis developing within 100 days of allogeneic haematopoietic (stem) cell transplantation. Acute GVHD occurs in up to 2/3 of HLA-identical siblings and is increasingly common as HLA mismatching increases, and occurs in 80% of marrow recipients.Clinical findings
• Hyperacute GVHD—fever, generalised erythroderma, desquamation with 2 weeks of transplantation.
• Acute GVHD—pruritic or painful rash, elevated liver enzymes with cholestatic jaundice; GI tract involvement is associated with diarrhoea, bleeding, painful bloating, and ileus.
Complications
Acute GVHD carries an increased risk of pneumonia and sterile effusions, hemorrhagic cystitis, thrombocytopenia, and anaemia.
Diagnosis
Lab—autoimmune cytopaenias, increased alkaline phosphatase, cholestasis, hypoalbuminaemia due to GVHD-associated leakage of protein into the gut, negative nitrogen balance, electrolyte imbalance due to diarrhoea.
Management
Primary prophylaxis with cyclosporin A (CSA), methotrexate, antithymocyte globulin (ATG). If Acute GVHD develops, corticosteroids, increase dose of CSA, add tacrolimus, ATG, and various monoclonal antibodies—none of which have proven clearly superior.