Aplastic anemia after liver transplantation for fulminant liver failure

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).

Section: Discussionsupporting

confidence: 58%

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

Umeda

Adachi

Watanabe

et al. 2002

“…SAA is a well-reported and common complication of liver transplantation for non-A, non-B, non-C hepatitis with reports of mortality rates as high as 40-50% during courses of immunosuppressive therapy, primarily due to infectious complications. 27,28 HSCT for SAA has been reported following liver and kidney transplantation. 29 However, successful allogeneic HSCT in pediatric patients developing SAA following orthotopic liver transplantation has been previously described only in case reports.…”

Section: Discussionmentioning

confidence: 99%

Hematopoietic stem-cell transplantation following solid-organ transplantation in children

Schechter

Gassas

Weitzman

et al. 2011

Reports of hematopoietic stem-cell transplantation (HSCT) following solid-organ transplantation have been described in adults mainly as case reports. These reports demonstrate feasibility but likely do not reflect true outcomes due to a positive reporting bias. We report herein the outcomes of all our pediatric recipients of allogeneic HSCT following previous solid-organ transplantation between 2000 and 2009. Four children were identified. Two patients underwent heart transplantation followed by cord-blood allogeneic HSCT for T-cell lymphoma/post transplant lymphoproliferative disease (PTLD) and two patients underwent liver transplantation followed by living-donor allogeneic HSCT for severe aplastic anemia (SAA). The mean time between transplants was 4.2 years (range 1.5-6 years). All patients engrafted; however, all patients died from 37 days to 1 year after HSCT. Causes of death included infections (n ¼ 2), multi-organ failure (n ¼ 1) and solid-organ graft rejection (n ¼ 1). Though three patients survived beyond day þ 100, multiple complications were observed including EBV re-activation followed by EBV-positive PTLD (n ¼ 1) and five episodes of severe infections. The patients transplanted for lymphoma did not have evidence of recurrence at last follow-up. Although feasibilty has been shown with this cohort, we conclude that allogeneic HSCT in immunosuppressed patients following solid-organ transplantation remains a very high risk procedure that results in severe morbidity and mortality in children.

“…1,2 This complication has been noted to be significantly more frequent among children than adults, and mortality is high. Clinical experience suggests that aplastic anemia is a complication of the underlying disease which led to hepatic failure, rather than due to the process of transplantation, as SAA does not generally occur following liver transplantation for other reasons.…”

mentioning

confidence: 99%

“…3 Outcomes for patients whose aplastic anemia was treated with immunosuppressive agents have varied. 1,2 Full hematologic recovery following immunosuppression has been reported, however time to recovery is often on the order of several months to years. 1,2 Sato et al 4 recently described a child successfully treated with growth factors and immunosuppression for SAA which developed following living-related orthotopic liver transplantation for FHF.…”

mentioning

confidence: 99%

“…However, others have reported mortality rates of 40-50% during courses of immunosuppressive therapy, due primarily to infectious complications. 1,2 In this report, we describe the 8-year follow-up of an 11-year-old boy who underwent orthotopic cadaveric liver transplantation at the age of 3.5 years for fulminant hepatic failure secondary to non-A, non-B, non-C hepatitis, followed by sibling donor BMT for SAA.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Successful bone marrow transplantation for severe aplastic anemia following orthotopic liver transplantation: long-term follow-up and outcome

Perkins

Neglia

Ramsay

et al. 2001

Summary:Severe aplastic anemia (SAA) is well described in children following liver transplantation for fulminant hepatic failure (FHF) secondary to non-A, non-B, non-C hepatitis, and is associated with a high mortality rate. Successful immunosuppressive treatment of SAA following liver transplantation has been reported, but death from infectious complications is not uncommon. We report the 8-year follow-up of a 3.5-year-old boy who underwent successful HLA-identical sibling donor bone marrow transplant for SAA 7 months following orthotopic liver transplant for non-A, non-B, non-C hepatitis. His post-bone marrow transplantation course was uneventful with no evidence of liver toxicity. Eight months following BMT he developed renal cell carcinoma metastatic to lymph nodes which was treated surgically. Six years following BMT he developed a mucoepidermoid carcinoma of the parotid gland also treated surgically. Despite these malignancies, he is currently well 8 years following liver and bone marrow transplantation, without signs of GVHD, growth failure or liver graft rejection. This is the first report of long-term follow-up of bone marrow transplantation for SAA following liver transplantation. The occurrence of two subsequent malignancies in this child underscores the need for close follow-up of future similar cases. Bone Marrow Transplantation (2001) 28, 523-526.