Abstract
Patients with hepatitis-associated aplastic anemia (HAA) who undergo living-donor liver transplantation (LDLT) have a poor prognosis with infections and bleeding complications. Rapid recovery of blood cells is critical for preventing these complications and improving the outcome. Immunosuppressive therapy (IST) combined with thrombopoietin receptor agonists is considered effective for aplastic anemia. However, there are no data on the benefits of adding thrombopoietin receptor agonists to IST for HAA. We present the case of a child with severe HAA who underwent LDLT, and who achieved rapid blood cell recovery with IST combined with romiplostim, a thrombopoietin receptor agonist. In addition, despite having undergone LDLT, the patient had no adverse events such as serious liver dysfunction or thrombosis. This case suggests that IST combined with thrombopoietin receptor agonists may be a promising treatment option for HAA patients undergoing LDLT.
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References
Alshaibani A, Dufour C, Risitano A, de Latour R, Aljurf M. Hepatitis-associated aplastic anemia. Hematol Oncol Stem Cell Ther. 2020. https://doi.org/10.1016/j.hemonc.2020.10.001 (Online ahead of print).
De Bruyne RM, Dhawan A. Bone marrow dysfunction following pediatric liver transplantation. Pediatr Transplant. 2005;9:423–6.
Young NS. Aplastic anemia. N Engl J Med. 2018;379:1643–56.
Townsley DM, Scheinberg P, Winkler T, Desmond R, Dumitriu B, Rios O, et al. Eltrombopag added to standard immunosuppression for aplastic anemia. N Engl J Med. 2017;376:1540–50.
Camitta BM, Thomas ED, Nathan DG, Gale RP, Kopecky KJ, Rappeport JM, et al. A prospective study of androgens and bone marrow transplantation for treatment of severe aplastic anemia. Blood. 1979;53:504–14.
Kojima S, Hibi S, Kosaka Y, Yamamoto M, Tsuchida M, Mugishima H, et al. Immunosuppressive therapy using antithymocyte globulin, cyclosporine, and danazol with or without human granulocyte colony-stimulating factor in children with acquired aplastic anemia. Blood. 2000;96:2049–54.
Delehaye F, Habes D, Dourthe ME, Bertrand Y, Michel G, Gaudichon J, et al. Management of childhood aplastic anemia following liver transplantation for nonviral hepatitis: a french survey. Pediatr Blood Cancer. 2020;67:28177.
Osugi Y, Yagasaki H, Sako M, Kosaka Y, Taga T, Ito T, et al. Antithymocyte globulin and cyclosporine for treatment of 44 children with hepatitis associated aplastic anemia. Haematologica. 2007;92:1687–90.
Mohseny AB, Eikema DA, Neven B, Kröger N, Shaw PJ, Damaj G, et al. 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 (SAAWP-EBMT). J Pediatr Hematol Oncol. 2020. https://doi.org/10.1097/MPH.0000000000001991.
Scheinberg P, Wu CO, Nunez O, Young NS. Predicting response to immunosuppressive therapy and survival in severe aplastic anaemia. Br J Haematol. 2009;144:206–16.
Fuhrer M, Rampf U, Baumann I, Faldum A, Niemeyer C, Janka-Schaub G, et al. Immunosuppressive therapy for aplastic anemia in children: a more severe disease predicts better survival. Blood. 2005;106:2102–4.
Chang MH, Kim KH, Kim HS, Jun HJ, Kim DH, Jang JH, et al. Predictors of response to immunosuppressive therapy with antithymocyte globulin and cyclosporine and prognostic factors for survival in patients with severe aplastic anemia. Eur J Haematol. 2010;84:154–9.
Locasciulli A, Bacigalupo A, Bruno B, Montante B, Marsh J, Tichelli A, et al. Hepatitis-associated aplastic anaemia: epidemiology and treatment results obtained in Europe. A report of The EBMT aplastic anaemia working party. Br J Haematol. 2010;149:890–5.
Dultz G, Kronenberger B, Azizi A, Mihm U, Vogl TJ, Sarrazin U, et al. Portal vein thrombosis as complication of romiplostim treatment in a cirrhotic patient with hepatitis C-associated immune thrombocytopenic purpura. J Hepatol. 2011;55:229–32.
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HY, YK, YS, and AM wrote the manuscript. YK, HN, YO, YH, NO, YS, YO, YS, and A.M. provided medical care for the patient. All authors commented on the manuscript and contributed to the final manuscript.
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The authors declare no conflicts of interest in association with the present study.
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Written informed consent for the off-label use of romiplostim was obtained from the parents.
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Yoshinari, H., Kawahara, Y., Niijima, H. et al. Rapid blood cell recovery with immunosuppressive therapy combined with romiplostim in a patient with very severe hepatitis-associated aplastic anemia who underwent liver transplantation. Int J Hematol 114, 524–527 (2021). https://doi.org/10.1007/s12185-021-03165-z
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DOI: https://doi.org/10.1007/s12185-021-03165-z