J Korean Soc Transplant.  2011 Dec;25(4):276-281. 10.4285/jkstn.2011.25.4.276.

Serongative Acute Hepatic Failure-associated Aplastic Anemia in Pediatric Liver Transplantation

Affiliations
  • 1Department of Surgery, Seoul National University College of Medicine, Seoul, Korea. gsleenj@hanmail.net
  • 2Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
  • 3Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
  • 4Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.

Abstract

Aplastic anemia (AA) is a rare complication of liver transplantation. The causes of AA have not yet been identified, and optimal treatment for AA after liver transplantation has not been firmly established. We experienced two cases of AA accompanied with fulminant hepatitis among 157 pediatric recipients (1.3%) and among 17 recipients of Korean Network of Organ Sharing (KONOS) status 1 (11.8%). The patients were a 16-year-old girl and a 3-year-old boy who had jaundice and lethargy due to non-A, non-B, non-C fulminant hepatitis. The girl underwent split liver transplantation involving the liver of a 24-year-old man, and the boy underwent an emergency living donor liver transplantation with a liver obtained from his 16-year-old cousin. Each transplantation procedure was uneventful. However, both patients were diagnosed with AA caused by thrombocytopenia and neutropenia at 140 and 26 days, respectively, after liver transplantation. The girl recovered completely after undergoing bone marrow transplantation and was followed up for 70 months. However, the boy was conservatively treated because of the development of hyperbilirubinemia and pyrexia. He died of multi-organ failure 74 days after liver transplantation. AA is not a rare complication of pediatric liver transplantation for fulminant hepatic failure. Therefore, AA must be suspected in pediatric cases of cytopenia even after liver transplantation. Our findings indicate bone marrow transplantation is the treatment of choice for AA even in cases where AA develops after liver transplantation.

Keyword

Liver transplantation; Aplastic anemia; Bone marrow transplantation; Pediatric liver transplantation; Fulminant hepatic failure

MeSH Terms

Adolescent
Anemia, Aplastic
Bone Marrow Transplantation
Emergencies
Fever
Hepatitis
Humans
Hyperbilirubinemia
Jaundice
Lethargy
Liver
Liver Failure, Acute
Liver Transplantation
Living Donors
Neutropenia
Preschool Child
Thrombocytopenia
Transplants
Young Adult

Figure

  • Fig. 1. Explant liver pathology of the patients suffered from fulminant hepatic failure. (A) Explant liver pathology of a 16-year-old girl suffered from fulminant hepatic failure (HE stain, ×200). (B) Explant liver pathology of a 3-year-old boy suffered from fulminant hepatic failure (HE stain, ×400). The explants liver shows submassive hepatic necrosis with diffuse lobular replacement by Herring ductules.

  • Fig. 2. Bone marrow biopsy of a 3-year-old boy suffered from aplastic anemia after liver transplantation due to fulminant hepatic failure. The cellularity is 0∼10% which is hypocellular with marked decrease of Trilineage hematopoiesis.

  • Fig. 3. Plotting of liver function test in a 3-year-old boy after liver transplantation for fulminant hepatic failure. He developed hyperbilirubinemia since 27 days after liver transplantation.


Reference

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