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How I manage aplastic anaemia in children.
Br J Haematol. 2012 Apr; 157(1):26-40.BJ

Abstract

Aplastic anaemia (AA) is a rare heterogeneous condition in children. 15-20% of cases are constitutional and correct diagnosis of these inherited causes of AA is important for appropriate management. For idiopathic severe aplastic anaemia, a matched sibling donor (MSD) haematopoietic stem cell transplant (HSCT) is the treatment of choice. If a MSD is not available, the options include immunosuppressive therapy (IST) or unrelated donor HSCT. IST with horse anti-thymocyte globulin (ATG) is superior to rabbit ATG and has good long-term results. In contrast, IST with rabbit ATG has an overall response of only 30-40%. Due to improvements in outcome over the last two decades in matched unrelated donor (MUD) HSCT, results are now similar to that of MSD HSCT. The decision to proceed with IST with ATG or MUD HSCT will depend on the likelihood of finding a MUD and the differing risks and benefits that each therapy provides.

Authors+Show Affiliations

Paediatric Haematopoietic Stem Cell Transplant Unit, Department of Adolescent and Paediatric Haematology and Oncology, Great North Children's Hospital, Royal Victoria Infirmary, Newcastle Upon Tyne, UK. sujith.samarasinghe@nuth.nhs.ukNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

22348483

Citation

Samarasinghe, Sujith, and David K H. Webb. "How I Manage Aplastic Anaemia in Children." British Journal of Haematology, vol. 157, no. 1, 2012, pp. 26-40.
Samarasinghe S, Webb DK. How I manage aplastic anaemia in children. Br J Haematol. 2012;157(1):26-40.
Samarasinghe, S., & Webb, D. K. (2012). How I manage aplastic anaemia in children. British Journal of Haematology, 157(1), 26-40. https://doi.org/10.1111/j.1365-2141.2012.09058.x
Samarasinghe S, Webb DK. How I Manage Aplastic Anaemia in Children. Br J Haematol. 2012;157(1):26-40. PubMed PMID: 22348483.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - How I manage aplastic anaemia in children. AU - Samarasinghe,Sujith, AU - Webb,David K H, Y1 - 2012/02/20/ PY - 2011/11/25/received PY - 2012/01/09/accepted PY - 2012/2/22/entrez PY - 2012/2/22/pubmed PY - 2013/3/26/medline SP - 26 EP - 40 JF - British journal of haematology JO - Br. J. Haematol. VL - 157 IS - 1 N2 - Aplastic anaemia (AA) is a rare heterogeneous condition in children. 15-20% of cases are constitutional and correct diagnosis of these inherited causes of AA is important for appropriate management. For idiopathic severe aplastic anaemia, a matched sibling donor (MSD) haematopoietic stem cell transplant (HSCT) is the treatment of choice. If a MSD is not available, the options include immunosuppressive therapy (IST) or unrelated donor HSCT. IST with horse anti-thymocyte globulin (ATG) is superior to rabbit ATG and has good long-term results. In contrast, IST with rabbit ATG has an overall response of only 30-40%. Due to improvements in outcome over the last two decades in matched unrelated donor (MUD) HSCT, results are now similar to that of MSD HSCT. The decision to proceed with IST with ATG or MUD HSCT will depend on the likelihood of finding a MUD and the differing risks and benefits that each therapy provides. SN - 1365-2141 UR - https://www.unboundmedicine.com/medline/citation/22348483/How_I_manage_aplastic_anaemia_in_children_ L2 - https://doi.org/10.1111/j.1365-2141.2012.09058.x DB - PRIME DP - Unbound Medicine ER -