Acute Hepatitic Onset of Liver GVHD Occurring 9 Months Post-transplant
The least common presentation of liver GVHD develops as an acute hepatitic onset which develops following tapering or cessation of immunosuppressive therapy (IS) or following donor lymphocyte infusions for a graft-versus-leukemia effect. The clinical features differ from the usual hepatic GVHD, arising concurrently with skin and gut aGVHD. Instead, the clinical presentation features rapidly rising aminotransferases (>20x normal) followed by hyperbilirubinemia. The progression of changes is an initial lobular hepatitis followed by marked cholestasis and bile duct damage. The differential diagnosis of acute hepatitic onset of GVHD includes a number of other viral infections and an unusual late post-HSCT chronic inflammatory and fibrosing hepatitis with many plasma cells and damaged bile ducts without attendent GVHD or infection. Since it resembles autoimmune hepatitis, we thus propose calling this condition “chronic alloimmnune hepatitis” (“CAIH”). Sporadic cases of cirrhosis that occur 5-15 years post-HSCT and are attributed to GVHD isolated to the liver may well be a late manifestation of CAIH.
KeywordsAcute hepatitic onset of hepatic GVHD Viral hepatitides and the differential diagnosis of liver GVHD Hepatic GVHD Bile duct damage with GVHD Cholestatic GVHD
- 15.Strasser SI, Sullivan KM, Myerson D, et al. Cirrhosis of the liver in long-term marrow transplant survivors. Blood. 1999;93(10):3259–66.Google Scholar
- 17.Assandri R, Serana F, Montanelli A. Development of PBC/SSc overlap syndrome in chronic GVHD patient: immunological implications in the presence of mitochondrial, nucleolar and spindle midzone autoantigens. Gastroenterology and hepatology from bed to bench. 2017;10(4):323–31.Google Scholar