Pediatric Acute Liver Failure & Aplastic Anemia
Aplastic anemia is most commonly seen with infections such as infectious
hepatitis, EBV, CMV, parvovirus B19, and HIV. However, there is a
correlation between I-PALF and increased risk of developing aplastic
anemia (AA) and early signs of bone marrow dysfunction. There are
similarities between AA and I-PALF as they are more immune-driven
processes involving CD8+ T-cells and high levels of inflammatory
cytokines. AA is a well-described condition that may develop
concomitantly with acute hepatitis (hepatitis-associated aplastic anemia
(HAAA)) or following liver transplant in patients with fulminant liver
failure. The first report of AA after ALF was in 1987 by Stock et al.
Further cases have described it primarily in pediatric patients with
I-PALF (Itterbeek et
al. 2002). Mechanistic studies of HAAA have described immunologic
dysregulation as the main pathogenesis: activated CD8+ T cells are
cytotoxic to myelpoietic bone marrow cells, T-cell clones are formed
early on in acute hepatitis, these clones attack similar target antigens
including hepatocytes and myeloid cells(Ikawa et al. 2013).