Successful hematopoietic stem cell transplantation for aplastic anemia following living-related liver transplantation

Abstract

Summary:A 1-year-old boy received a living-related liver transplantation (LRLT) from his HLA-haploidentical father to treat acute liver failure following non-A, non-B, non-C hepatitis. He subsequently developed pancytopenia and was diagnosed with aplastic anemia (AA). He was platelet transfusion dependent and developed two episodes of life-threatening intracranial hemorrhage despite immuno-suppressive therapy consisting of cyclosporin A, antithymocyte globulin, and anabolic steroids. He received combined hematopoietic stem cell transplantation (hSCT) with cord blood and bone marrow from an HLA-matched sibling. Conditioning consisted of cyclophosphamide (CY) 200 mg/kg and 7 Gy total lymphoid irradiation (TLI). Marrow engraftment was prompt and there was no significant graft-versus-host disease (GVHD).

Parvovirus Associated Fulminant Hepatic Failure and Aplastic Anemia Treated Successfully With Liver and Bone Marrow Transplantation. A Report of Two Cases

“…Although multiple drugs have reportedly been used for the management of SOS, none is recommended as standard treatment at this time. Development of SOS has been reported in only one patient who underwent both LT and BMT for FHF and associated AA (11). This is also the only reported case besides the two in this report where the allograft was obtained from a living donor.…”

“…More recently, bone marrow transplantation (BMT) has been utilized with excellent outcomes in these patients. An extensive literature search identified only 10 cases that underwent BMT after LT for FHF and AA (5)(6)(7)(8)(9)(10)(11)(12)(13). No causative agent for the FHF or AA has been identified in any of these cases.…”

Liver Transplantation Followed by Allogeneic Hematopoietic Stem Cell Transplantation for Atypical Mevalonic Aciduria

“…We felt that a RIC regimen (16) would be sufficient for engraftment and associated with fewer complications, especially considering the nutritional debilitation the patient exhibited and the intrinsic toxicity mevalonic kinase deficiency imposes on leukocytes. There are few reports of allogeneic peripheral blood stem cell transplants being performed after liver transplantation (22)(23)(24)(25). Our case differs from these in her unique diagnosis as well the lack of experience with a RIC regimen in this disorder.…”

Hepatitis-associated Aplastic Anemia Presenting as a Familial Bone Marrow Failure Syndrome

“…11,14,22 Our cases underscore the importance of thorough evaluation of sibling donors in FHF-associated aplastic anemia for HSCT. Matched related donor for HSCT in SAA gives superior results and is the preferred choice in aplastic anemia; however, it is essential to rule out the possibility of transplanting dysfunctional bone marrow from a yet unaffected sibling.…”