Abstract
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.
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References
Strasser SI, Shulman HM, Flowers ME, et al. Chronic graft-versus-host disease of the liver: presentation as an acute hepatitis. Hepatology. 2000;32(6):1265–71.
Ma SY, Au WY, Ng IO, et al. Hepatitic graft-versus-host disease after hematopoietic stem cell transplantation: clinicopathologic features and prognostic implication. Transplantation. 2004;77(8):1252–9.
Baniak NM, Kanthan R. Autoimmune-like hepatitis: a “Hepatitic” manifestation of chronic graft versus host disease in post-stem cell transplant. Int J Surg Pathol. 2016;24(2):146–52.
Akpek G, Boitnott JK, Lee LA, et al. Hepatitic variant of graft-versus-host disease after donor lymphocyte infusion. Blood. 2002;100(12):3903–7.
Narita A, Muramatsu H, Takahashi Y, et al. Autoimmune-like hepatitis following unrelated BMT successfully treated with rituximab. Bone Marrow Transplant. 2012;47(4):600–2.
Koyama D, Ito M, Yokohata E, et al. Autoimmune-like hepatitis after allogeneic hematopoietic stem cell transplantation: humoral hepatic GvHD. Bone Marrow Transplant. 2017;52(1):151–3.
Granito A, Stanzani M, Muratori L, et al. LKM1-positive type 2 autoimmune hepatitis following allogenic hematopoietic stem-cell transplantation. Am J Gastroenterol. 2008;103(5):1313–4.
Quaranta S, Shulman H, Ahmed A, et al. Autoantibodies in human chronic graft-versus-host disease after hematopoietic cell transplantation. Clin Immunol. 1999;91(1):106–16.
Carre M, Thiebaut-Bertrand A, Larrat S, et al. Fatal autochthonous fulminant hepatitis E early after allogeneic stem cell transplantation. Bone Marrow Transplant. 2017;52(4):643–5.
Willemse SB, Bezuur DL, Blom P, et al. Hepatitis E virus infection and hepatic GvHD in allogeneic hematopoietic stem cell transplantation recipients. Bone Marrow Transplant. 2017;52(4):622–4.
Hill JA, Myerson D, Sedlak RH, Jerome KR, Zerr DM. Hepatitis due to human herpesvirus 6B after hematopoietic cell transplantation and a review of the literature. Transpl Infect Dis. 2014;16(3):477–83.
Rees GM, Sarmiento JI, Myerson D, Coen D, Meyers JD, Mcdonald G. Cytomegalovirus hepatitis in marrow transplant patients: clinical, histologic and histochemical analysis. Gastroenterology. 1990;98:A470.
Snover DC, Hutton S, Balfour HH Jr, Bloomer JR. Cytomegalovirus infection of the liver in transplant recipients. J Clin Gastroenterol. 1987;9(6):659–65.
Larson AM, McDonald GB. Hepatobillary infections after solid organ or hematopoietic cell transplantation. In: Ljungman P, Snydman DR, Boeckh M, editors. Transplant infections. 4th ed. Cham: Springer International Publishing AG; 2016. p. 653–73.
Strasser SI, Sullivan KM, Myerson D, et al. Cirrhosis of the liver in long-term marrow transplant survivors. Blood. 1999;93(10):3259–66.
A Xhaard, P Nahon, M Robin, C Baudry, L Ades, R Peffault de Latour, G Socié, (2012) Hepatic GVHD leading to cirrhosis after allogeneic hematopoietic SCT. Bone Marrow Transplantation. 47(11):1484–5.
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.
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Shulman, H.M., Loeb, K.R. (2019). Acute Hepatitic Onset of Liver GVHD Occurring 9 Months Post-transplant. In: Yeung, C., Shulman, H. (eds) Pathology of Graft vs. Host Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-42099-8_16
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DOI: https://doi.org/10.1007/978-3-319-42099-8_16
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