Journal of Hepatology
Volume 48, Issue 1 , Pages 68-73, January 2008

Evolution in the management of acute liver failure-associated aplastic anaemia in children: A single centre experience☆☆☆

  • Nedim Hadžić

      Affiliations

    • Institute of Liver Studies, King’s College London School of Medicine at King’s College, London, UK
    • Corresponding Author InformationCorresponding author. Address: Paediatric Liver Centre, Variety Club Children’s Hospital, King’s College Hospital, Denmark Hill, London SE5 9RS, UK. Tel.: +44 0 2032994120; fax: +44 0 2032994228.
    • ,
  • Susan Height

      Affiliations

    • Department of Haematology, King’s College London School of Medicine at King’s College, London, UK
    • ,
  • Sarah Ball

      Affiliations

    • Department of Haematology, St. George’s Hospital, London, UK
    • ,
  • Mohamed Rela

      Affiliations

    • Institute of Liver Studies, King’s College London School of Medicine at King’s College, London, UK
    • ,
  • Nigel D. Heaton

      Affiliations

    • Institute of Liver Studies, King’s College London School of Medicine at King’s College, London, UK
    • ,
  • Paul Veys

      Affiliations

    • Bone Marrow Transplant Unit, Great Ormond Street Hospital for Children, London, UK
    • ,
  • Giorgina Mieli-Vergani

      Affiliations

    • Institute of Liver Studies, King’s College London School of Medicine at King’s College, London, UK

Received 1 May 2007; received in revised form 22 July 2007; accepted 1 August 2007. published online 25 October 2007.

Associate Editor: P.-A Clavien

Background/Aims

Bone marrow failure (BMF) is a potentially life-threatening complication of acute liver failure (ALF).

Methods

To investigate prevalence and evolving management of BMF associated with ALF, we reviewed all cases seen in our centre over 17 years. BMF was classified as: (a) bone marrow hypoplasia, (b) severe aplastic anaemia (SAA) and (c) very severe aplastic anaemia (VSAA), using standard criteria. We compared outcomes in children receiving: (1) medical treatment only with or without immunomodulation (anti-lymphocyte globulin, calcineurin inhibitors, G-CSF); (2) medical treatment with or without immunomodulation plus liver transplantation (LT); (3) haematopoietic stem cell transplantation (HSCT).

Results

Of 213 patients with ALF, 20 [(9.4%); 14 (70%) boys] developed BMF after a median of 1 month (range, 0.5 to 7). Seven had VSAA, 7 SAA and 6 bone marrow hypoplasia. Five children were treated medically, including 3 by immunomodulation; 10 (50%) received LT, with immunomodulation in 6; 5 (25%) received HSCT, in one after LT. Four (20%) children died, only one as consequence of AA. There was no difference in recovery, complication rates or outcome among the three groups.

Conclusions

Aggressive management of ALF-associated AA, including immunomodulation, HSCT and LT, is successful in most cases. HSCT has the advantage of removing the risk of late clonal disorders.

Abbreviations: ALF, acute liver failure, BMF, bone marrow failure, AA, aplastic anaemia, SAA, severe aplastic anaemia, VSAA, very severe aplastic anaemia, G-CSF, granulocyte-colony stimulating factor, ATG, anti-thymocyte globulin, ALG, anti-lymphocyte globulin, HSCT, haematopoietic stem cell transplantation, MOF, multiorgan failure, LT, liver transplant, GvHD, graft-versus-host disease, CR, chronic rejection

Keywords: Liver transplantation, Bone marrow failure, Aplastic anaemia, Haemopoietic stem cell transplantation

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☆ The authors declare that they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript.

☆☆ This work was presented as a poster at the Annual Meeting of the American Association for the Study of Liver Disease (AASLD) in Boston, USA, 26–31 October 2006.

PII: S0168-8278(07)00565-X

doi:10.1016/j.jhep.2007.08.017

Journal of Hepatology
Volume 48, Issue 1 , Pages 68-73, January 2008

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