Management of childhood aplastic anemia following liver transplantation for nonviral hepatitis: A French survey. Issue 4 (11th January 2020)

• Record Type: Journal Article
• Authors: Delehaye, Fanny; Habes, Dalila; Dourthe, Marie‐Emilie; Bertrand, Yves; Michel, Gerard; Gaudichon, Jérémie; Debray, Dominique; Nelken, Brigitte; Pasquet, Marlène; Blanche, Stéphane; Leblanc, Thierry
• Is Part Of: Pediatric blood & cancer. Volume 67:Issue 4(2020)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2020-01-11
• Subjects: aplastic anemia -- children -- hepatitis‐associated aplastic anemia -- liver transplantation; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.28177
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Background: Hepatitis‐associated aplastic anemia (AA) is a rare syndrome combining acute hepatitis of variable severity and AA. Hepatitis may be severe enough to require urgent liver transplantation (LT). Herein, we describe clinical presentation and management of a cohort of pediatric patients diagnosed with AA after undergoing LT for nonviral hepatitis. Methods: To describe this rare clinical situation, we performed a national survey and identified nine children treated for AA following LT during the last 10 years in France. Results: All patients were treated first for hepatic failure with urgent LT. AA was diagnosed with a median delay of 34 days [21‐200] from the diagnosis of hepatitis. Seven children were treated with antithymocyte globulin/cyclosporine, one with CSA alone and one received bone marrow transplantation. At the last visit (median follow‐up: 4 years), outcomes were excellent: all patients were alive and in hematological remission (complete remission: 7; partial remission: 2). Immunosuppressive therapy was pursued in all patients due to the liver transplant. No unusual toxicities were reported. Conclusion: AA after LT is considered a therapeutic challenge. Nevertheless, hematological outcome is good using a standard immunosuppressive approach.