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Myelodisplasias

Improvement of criteria for refractory cytopenia with multilineage dysplasia according to the WHO classification based on prognostic significance of morphological features in patients with refractory anemia according to the FAB classification

Abstract

In the criteria of refractory cytopenia with multilineage dysplasia (RCMD) according to the WHO (World Health Organization) classification, the frequency threshold concerning dysplasia of each lineage was defined as 10%. To predict overall survival (OS) and leukemia-free survival (LFS) for patients with refractory anemia (RA) according to the French-American-British (FAB) classification, we investigated prognostic factors based on the morphological features of 100 Japanese and 87 German FAB-RA patients, excluding 5q-syndrome. In the univariate analysis of all patients, pseudo-Pelger–Huet anomalies 10% (Pelger+), micromegakaryocytes 10% (mMgk+), dysgranulopoiesis (dys G) 10% and dysmegakaryopoiesis (dys Mgk) 40% were unfavorable prognostic factors for OS and LFS (OS; P<0.001, LFS; P<0.001). The prognostic effects of the morphological features were similar in both Japanese and German patients. However, dys Mgk 10% was not correlated with OS and LFS. In the multivariate analysis, mMgk+ and dys Mgk40% were adverse prognostic factors for OS for all patients, and dys G 10% and dys Mgk40% were adverse prognostic factors for LFS for all patients. On the basis of the present analysis, we propose the following modified morphological criteria for RCMD. Modified RCMD should be defined as FAB-RA, excluding 5q-syndrome with dys G 10%, dys Mgk40% or mMgk+.

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References

  1. Goasguen JE, Bennett JM . Classification and morphologic features of the myelodysplastic syndromes. Semin Oncol 1992; 19: 4–13.

    CAS  Google Scholar 

  2. Ganser A, Hoelzer D . Clinical course of myelodysplastic syndromes. Hematol Oncol Clin North Am 1992; 6: 607–617.

    Article  CAS  Google Scholar 

  3. Koeffler HP . Introduction: myelodysplastic syndromes. Semin Hematol 1996; 33: 87–94.

    CAS  Google Scholar 

  4. Hofmann WK, Ottmann OG, Ganser A, Hoelzer D . Myelodysplastic syndromes: clinical features. Semin Hematol 1996; 33: 177–185.

    CAS  Google Scholar 

  5. Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997; 89: 2079–2088.

    CAS  Google Scholar 

  6. Jaffe ES, Harris NL, Stein H, Vardiman JW (eds.) World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. IARC Press: Lyon, France, 2001.

    Google Scholar 

  7. Germing U, Gattermann N, Strupp C, Aivado M, Aul C . Validation of the WHO proposals for a new classification of primary myelodysplastic syndromes: a retrospective analysis of 1600 patients. Leuk Res 2000; 24: 983–992.

    Article  CAS  Google Scholar 

  8. Rosati S, Mick R, Xu F, Stonys E, Le Beau MM, Larson R et al. Refractory cytopenia with multilineage dysplasia: further characterization of an ‘unclassifiable’ myelodysplastic syndrome. Leukemia 1996; 10: 20–26.

    CAS  Google Scholar 

  9. Lorand-Metze I, Pinheiro MP, Ribeiro E, de Paula EV, Metze K . Factors influencing survival in myelodysplastic syndromes in a Brazilian population: comparison of FAB and WHO classifications. Leuk Res 2004; 28: 587–594.

    Article  Google Scholar 

  10. Matsuda A, Jinnai I, Yagasaki F, Kusumoto S, Minamihisamatsu S, Honda S et al. Refractory anemia with severe dysplasia: clinical significance of morphological features in refractory anemia. Leukemia 1998; 12: 482–485.

    Article  CAS  Google Scholar 

  11. Matsuda A, Jinnai I, Yagasaki F, Kusumoto S, Murohashi I, Bessho M et al. New system for assessing the prognosis of refractory anemia patients. Leukemia 1999; 13: 1727–1734.

    Article  CAS  Google Scholar 

  12. Matsuda A, Germing U, Jinnai I, Misumi M, Kuendgen A, Knipp S et al. Difference in clinical features between Japanese and German patients with refractory anemia in myelodysplastic syndromes. Blood 2005; 106: 2633–2640.

    Article  CAS  Google Scholar 

  13. Germing U, Gattermann N, Aivado M, Hildebrandt B, Aul C . Two types of acquired idiopathic sideroblastic anaemia (AISA): a time-tested distinction. Br J Haematol 2000; 108: 724–728.

    Article  CAS  Google Scholar 

  14. Nosslinger T, Reisner R, Koller E, Gruner H, Tuchler H, Nowotny H et al. Myelodysplastic syndromes, from French-American-British to World Health Organization: comparison of classifications on 431 unselected patients from a single institution. Blood 2001; 98: 2935–2941.

    Article  CAS  Google Scholar 

  15. Kuriyama K, Tomonaga M, Matsuo T, Ginnai I, Ichimaru M . Diagnostic significance of detecting pseudo-Pelger–Huet anomalies and micro-megakaryocytes in myelodysplastic syndrome. Br J Haematol 1986; 63: 665–669.

    Article  CAS  Google Scholar 

Download references

Acknowledgements

Supported in part by a Grant-in-Aid for Scientific Research from the Japan Society for the Promotion of Science (no. 16639013) (IJ) and Kompetenznetzwerk ‘Akute und Chronische Leukämien’ des Bundesforschungsministeriums.

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Correspondence to A Matsuda.

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Matsuda, A., Germing, U., Jinnai, I. et al. Improvement of criteria for refractory cytopenia with multilineage dysplasia according to the WHO classification based on prognostic significance of morphological features in patients with refractory anemia according to the FAB classification. Leukemia 21, 678–686 (2007). https://doi.org/10.1038/sj.leu.2404571

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