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graft versus host disease (GVHD)

Etiology: - hematopoietic stem cell transplantation (most common) - blood transfusion in immunocompromised host [2] Pathology: 1) 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 [2] 2) chronic phase (months to 1 year after transplantation) a) results form donor immunocompetent lymphocytes that develop within the recipient b) lymphocytes in proximity to dyskeratotic keratinocytes (satellite cell necrosis) c) cutaneous infiltrate consists of predominantly CD8 cells Clinical manifestations: 1) acute phase a) 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 b) nausea, vomiting, non-bloody diarrhea [4] c) liver function abnormalities 2) chronic phase a) 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 b) liver function abnormalities - primary biliary cirrhosis-like syndrome c) eyes: cataracts, dry & red eyes 4) oral mucosa: decreased salivation, tooth decay, oral ulcers e) respiratory: bronchiolitis obliterans, sinus & lung infection f) immunologic: immunodeficiency, bacterial & viral infection g) muscle: myositis h) gastrointestinal: malabsorption, abdominal pain i) hematopoietic: pancytopenia j) reproductive: decreased libido, vaginal dryness k) malignancies in areas of previous GVHD Management: 1) life-threatening acute graft vs host disease is treated with high-dose glucocorticoids [2] 2) cyclosporine & methotrexate +/- prednisone reduces incidence of GVHD to ~20% 3) chronic GVHD requires treatment with cyclosporine & prednisone for 6 months to 3 years - ruxolitinib for glucocorticoid-refractory chronic GVHD [3] 4) other useful agents a) thalidomide b) azathioprine c) psoralen + ultraviolet A (PUVA)

Related

bone marrow transplantation (BMT)

Specific

graft-versus-leukemia (GVL)

General

autoimmune disease

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 177-78
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 17. American College of Physicians, Philadelphia 1998, 2015
  3. Zeiser R, Polverelli N, Ram R et al Ruxolitinib for Glucocorticoid-Refractory Chronic Graft-versus-Host Disease. N Engl J Med 2021; 385:228-238. July 15. PMID: 34260836 https://www.nejm.org/doi/full/10.1056/NEJMoa2033122
  4. NEJM Knowledge+