Immunotherapy for severe aplastic anemia following orthotopic liver transplantation in children

Abstract

Severe aplastic anemia is a well-recognized complication of fulminant non-A, non-B, and non-C hepatitis requiring orthotopic liver transplantation. The first line of therapy for cure in the treatment of aplastic anemia is a histocompatible bone marrow transplant. Immunosuppressive therapy is also effective if a histocompatible donor is not available. We describe two children who developed severe aplastic anemia following orthotopic liver transplant who achieved bone marrow recovery with a single course of anti-thymocyte globulin, solumedrol, and adjustments to their immunosuppressive therapy for prevention of liver allograft rejection.

Hematopoietic Stem Cell Transplantation for Hepatitis-associated Aplastic Anemia Following Liver Transplantation for Nonviral Hepatitis: A Retrospective Analysis and a Review of the Literature by the Severe Aplastic Anemia Working Party of the European Society for Blood and Marrow Transplantation

“…IST as an alternative treatment option for HAAA after LT for nonviral hepatitis was previously reported successful by several case reports [26][27][28][29] with comparable outcomes to HSCT. 10 However, Delehaye et al 6 recently reported excellent outcomes of IST for childhood HAAA after LT for nonviral hepatitis.…”

Sequential transplantation and implications for clinical management: OLT followed by HCT and consequent RT in a pediatric patient

“…[1][2][3][4][5][6][7][8][9][10]19 Table 1 summarizes the various reports published on patients with SAA complicating non-A-E hepatitis. [1][2][3][5][6][7][20][21][22][23][24][25][26] It is hypothesized that the complication of SAA is likely associated with the original mechanism of hepatic failure, rather than due to the transplantation, 6 and is likely mediated by immunopathologic mechanisms and inappropriate cytotoxic T-cell activation, as evidenced by response to immunotherapy. 3,4,9 This disease association has a high mortality rate, estimated in the 30%-50% range.…”

“…It has been well documented that SAA is a complication of non‐A‐E hepatitis in children, with success reported both in immunotherapy treatment and undergoing HCT after resolution of hepatitis . Table summarizes the various reports published on patients with SAA complicating non‐A‐E hepatitis . It is hypothesized that the complication of SAA is likely associated with the original mechanism of hepatic failure, rather than due to the transplantation, and is likely mediated by immunopathologic mechanisms and inappropriate cytotoxic T‐cell activation, as evidenced by response to immunotherapy .…”

Treatment of hepatitis‐associated aplastic anemia with high‐dose cyclophosphamide

“…Most cases of HAA are seronegative hepatitis without demonstrable autoantibodies 20. Another known association of hepatitis to aplastic anemia is liver transplantation 21–23. There is no consistent viral pathogen that links hepatitis and aplastic anemia.…”