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

Abstract

Hepatitis-associated aplastic anemia (HAAA) has been reported in 23% to 33% of patients who received orthotopic liver transplantation (LT) for acute liver disease of unknown origin (nonviral hepatitis). In this situation, hematopoietic stem cell transplantation (HSCT) might be a curative option. Here the authors report on 6 patients who received HSCT after LT for nonviral HAAA hepatitis. The outcomes were interpreted in the context of recently reported immune suppressive therapy (IST) outcomes in 8 patients with HAAA and to HSCT outcomes in patients with HAAA who recovered from hepatitis without undergoing LT. All patients transplanted by using HLA-identical sibling donors (3 of 6) were alive and had normal liver function and hematopoiesis without graft versus host disease. Both patients receiving bone marrow from a matched unrelated donor (MUD) experienced extensive graft versus host disease that was fatal for one patient. Thereby, the authors conclude that HSCT can be considered as a first-choice treatment for this category of patients when HLA-identical donors are available. When no HLA-identical donor is available, IST should be applied as HSCT with other donor sources might be reserved for IST nonresponders or poor responders.

Successful treatment with HLA-matched peripheral hematopoietic stem cell transplantation for very severe hepatitis-associated aplastic anemia complicated with multidrug-resistant bacterial and fungal infections: A case report

“…It has been suggested to take earlier approaches for patients when HAAA is developing (11). HSCT is preferred over immune suppressive therapy if an HLA-identical donor is available (12). Mori et al showed that allogeneic HSCT is safe with a low rate of transplant-related mortality (3).…”

Hepatitis-Associated Aplastic Anemia: Etiology, Clinical Characteristics and Outcome

“…1,2,11,13 The definitive treatment options for HAA are IST and HSCT, as in the case of acquired AA. [3][4][5][6]8,10,13 In children with acquired AA, HSCT has been recommended as the first-line therapy when HLAmatched related donor is available. 15,18 Multiple studies have reported 5-year survival rates of 90% or more in children who underwent HSCT with a matched related donor.…”

“…The course of hepatitis is unpredictable but AA is lethal if left untreated 1,2,11,13. The definitive treatment options for HAA are IST and HSCT, as in the case of acquired AA 3–6,8,10,13. In children with acquired AA, HSCT has been recommended as the first-line therapy when HLA-matched related donor is available 15,18.…”

“…[4][5][6] Indeed, AA has also been reported after orthotopic liver transplantation undertaken for acute fulminant non-A, non-B, and non-C hepatitis. [6][7][8] Although some viral hepatitis agents have been identified in the etiology, the cause is unknown in most cases. 4,6,[9][10][11] Bone marrow failure leading to AA is generally severe in cases of HAA and fatal if left untreated.…”

“…Immunosuppressive treatment (IST) with cyclosporine and antithymocyte globulin (ATG) and hematopoietic stem cell transplantation (HSCT) have been successfully used for treating AA. 3,6,8,[11][12][13] In this study, we investigated the clinical and laboratory characteristics, possible causes, treatment and outcome of HAA in children.…”