graft versus host disease (GVHD)

Etiology

• hematopoietic stem cell transplantation (most common)
• blood transfusion in immunocompromised host

Pathology

• acute phase (7-21 days after transplantation)
  • attack of donor immunocompetent T-lymphocytes & null lymphocytes against host histocompatibility antigens
  • intestine, skin & liver sinusoids identified as targets
• chronic phase (months to 1 year after transplantation)
  • results form donor immunocompetent lymphocytes that develop within the recipient
  • lymphocytes in proximity to dyskeratotic keratinocytes (satellite cell necrosis)
  • cutaneous infiltrate consists of predominantly CD8 cells

Clinical manifestations

• acute phase
  • skin manifestations
    • pruritus
    • numbness or pain of the palms & soles
    • erythematous macular-papular eruption on the trunk palms & soles
    • blisters that may resemble toxic epidermal necrolysis
  • nausea, vomiting, non-bloody diarrhea
  • liver function abnormalities
• chronic phase
  • skin manifestations
    • lichenoid reaction
      • oral & cutaneous lesions resembling lichen planus
      • coalescing violaceous papules on skin
      • white reticulated patches on the buccal mucosa
    • cutaneous sclerosis (generalized skin thickening)
    • poikilodermatous-reticulated lesions
    • scarring alopecia
  • liver function abnormalities
    • primary biliary cirrhosis-like syndrome
  • eyes: cataracts, dry & red eyes
• oral mucosa: decreased salivation, tooth decay, oral ulcers
  • respiratory: bronchiolitis obliterans, sinus & lung infection
  • immunologic: immunodeficiency, bacterial & viral infection
  • muscle: myositis
  • gastrointestinal: malabsorption, abdominal pain
  • hematopoietic: pancytopenia
  • reproductive: decreased libido, vaginal dryness
  • malignancies in areas of previous GVHD

Management

• life-threatening acute graft vs host disease is treated with high-dose glucocorticoids
• cyclosporine & methotrexate +/- prednisone reduces incidence of GVHD to ~20%
• chronic GVHD requires treatment with cyclosporine & prednisone for 6 months to 3 years
  • ruxolitinib for glucocorticoid-refractory chronic GVHD
• other useful agents
  • thalidomide
  • azathioprine
  • psoralen + ultraviolet A (PUVA)

More general terms

• autoimmune disease

More specific terms

• graft-versus-leukemia (GVL)

Additional terms

• bone marrow transplantation (BMT)