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Diagnosis of acquired bone marrow failure syndrome during childhood using the 2008 World Health Organization classification system

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Abstract

Distinguishing hypoplastic myelodysplastic syndrome from aplastic anemia (AA) is challenging. In the present study, Japanese and Chinese pediatric hematologists and pathologists conducted a joint review of bone marrow (BM) smears and trephine biopsies in 100 children with acquired BM failure syndrome, using the criteria proposed in the 2008 edition of the World Health Organization classification of hematopoietic and lymphoid tissues. The final consensus for the diagnoses of 100 children was AA in 29 patients, refractory cytopenia of childhood (RCC) in 58 patients, and refractory cytopenia with multilineage dysplasia (RCMD) in 13 patients. No significant differences between Japanese and Chinese children were found with regards to clinical and laboratory findings, or the distribution of diagnoses. Patients with RCC/RCMD showed milder disease severity and BM hypocellularity than those with AA. To establish the provisional entities for RCC, it is essential to prospectively compare the clinical outcomes between AA and RCC groups in a large number of patients.

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References

  1. McCahon E, Tang K, Rogers PCJ, McBride ML, Schultz KR. The impact of Asian descent on the incidence of acquired severe aplastic anaemia in children. Br J Haematol. 2003;121:170–2.

    Article  PubMed  Google Scholar 

  2. Nakao S, Yamaguchi M, Takamatsu H, Takami A, Chuhjo T, Ueda M, et al. Relative erythroid hyperplasia in the bone marrow at diagnosis of aplastic anaemia: a predictive marker for a favourable response to cyclosporine therapy. Br J Haematol. 1996;92:318–23.

    Article  PubMed  CAS  Google Scholar 

  3. Baumann I, Niemeyer CM, Bennett JM, Shannon K. WHO classification of tumors of haematopoietic and lymphoid tissues. In: Swerdlow SH, Campo E, Harris Nl, Jaffe ES, Pileri SA, Stein H, et al., editors. Childhood myelodysplastic syndrome; 2008. p. 104–7

  4. International Agranulocytosis and Aplastic Anemia Study Group. Incidence of aplastic anemia: the relevance of diagnostic criteria. Blood. 1987;70:1718–21.

    Google Scholar 

  5. Camitta BM, Thomas ED, Nathan DG, Santos G, Gordon-Smith EG, Gale RP. Severe aplastic anemia: a prospective study of the effect of early marrow transplantation on acute mortality. Blood. 1976;48:63–9.

    PubMed  CAS  Google Scholar 

  6. Bennett JM, Orazi A. Diagnostic criteria to distinguish hypocellular acute myeloid leukemia from hypocellular myelodysplastic syndromes and aplastic anemia: recommendations for a standardized approach. Haematologica. 2009;94:264–8.

    Article  PubMed  Google Scholar 

  7. Rajnoldi AC, Fenu S, Kerndrup G, van Wering ER, Niemeyer CM, Baumann I, for the European Working Group on Myelodysplastic Syndromes in Childhood (EWOG-MDS). Evaluation of dysplastic features in myelodysplastic syndromes: experience from the morphology group of the European Working Group of MDS in Childhood (EWOG-MDS). Ann Hematol. 2005;84:429–33.

    Article  Google Scholar 

  8. Führer 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.

    Article  PubMed  Google Scholar 

  9. Yoshimi A, Baumann I, Führer M, Bergsträsser E, Göbel U, Sykora KW, Klingebiel T. Immunosuppressive therapy with anti-thymocyte globulin and cyclosporine A in selected children with hypoplastic refractory cytopenia. Haematologica. 2007;92:397–400.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

This work was supported in part by grants from China health ministry subordinates hospital clinical subject key programs in 2010–2012.

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The authors reported no potential conflicts of interest.

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Correspondence to Xiaofan Zhu.

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Yang, W., Zhang, P., Hama, A. et al. Diagnosis of acquired bone marrow failure syndrome during childhood using the 2008 World Health Organization classification system. Int J Hematol 96, 34–38 (2012). https://doi.org/10.1007/s12185-012-1092-z

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  • DOI: https://doi.org/10.1007/s12185-012-1092-z

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