Myelodysplastic syndromes (MDS) are a group of heterogeneous clonal stem cell disorders characterized by ineffective hematopoiesis with dysplastic changes in one or more myeloid cell lines and increased risk of progression to acute leukemia. The current diagnosis criteria include the morphology of peripheral blood (PB) and bone marrow (BM), bone marrow biopsy and cytogenetic exam. Material and method. For this study, we have analyzed 33 patients diagnosed with lower-risk MDS (IPSS 0 and intermediate-1) according to the World Health Organization (WHO) classification (2001) between 2008 and 2012. The diagnosis was confirmed by blood cell counts, bone marrow (aspirate and biopsy) exam and cytogenetic exam. Other causes of cytopenia or dysplastic changes were excluded. Results. The types of MDS according to the WHO classification were: nine patients with refractory anemia (RA) (27.27%), sixteen patients with refractory anemia with ringed sideroblasts (RARS) (48.48%), and eight patients with refractory cytopenia with multilineage dysplasia (RCMD) (24.24%) out of which two with refractory cytopenia with multilineage dysplasia with ringed sideroblasts (RCMD-RS). Cytogenetic exam was performed in all patients, but analyzable metaphases for cytogenetic exam were obtained only from twenty five patients. The patients who did not have analyzable metaphases on cytogenetic exam were considered low risk if: they had only one cytopenia and the percent of bone marrow blasts was less than 5%. For all patients who had analyzable metaphases at cytogenetic exam, the International Prognostic Scoring System (IPSS) and Revised International Prognostic Scoring System (R-IPSS) scores were determined, and their survival and the death leading events were observed. According to IPPS (1997), the cytogenetic exam was good in 17 cases, intermediate in 1 case and poor in 7 cases. The IPSS score was low in 13 cases and intermediate-1 in 12 cases. According to R-IPSS, cytogenetic exams had been very good and good in 17 cases, intermediate in 1 case, poor in 6 cases and very poor in 1 case. R-IPSS was very low and low in 17 cases and intermediate and high in 8 cases. Conclusions. This new R-IPSS score at diagnosis allows a more accurate classification of patients into risk groups and thus enables risk adapted therapy
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1. Bennett JM Catovsky D Daniel MT Flandrin G Galton DA Gralnick HR et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 1982 51:189-199.
2. Cazzola M Malcovati L: Myelodysplastic syndromes -coping with ineffective hematopoiesis. N Engl J Med 2005 352: 536-538.
3. Vardiman JW Harris NL Brunning RD: The new World Health Organization classification of myeloid neoplasms: Blood 2002 100:2292-2302.
4. Fenaux P: Myelodysplastic syndromes: from pathogenesis and prognosis to treatment. SeminHematol. 2004;41:6-12.
5. Greenberg P Cox C LeBeau MM Fenaux P Morel P Sanz G: International Scoring System for Evaluating Prognosis in Myelodysplastic Syndromes. Blood 1997; 89:2079-2088.
6. Greenberg P Tuechler H Schanz J Sanz G Solé F Bennett J et al: Revised International Prognostic Scoring System for Myelodysplastic Syndromes. Blood 2012 120: 2454-2465.
7. Swansbury J: Methods in Molecular Biology TMCancer Cytogenetics Methods and Protocols.Humana 2003;220: 43-56.
8. Shaffer LG Slovak ML Campbell JL: An International System for Human Cytogenetic Nomenclature 2009.
9. Nowell PC. Chromosome abnormalities in myelodysplastic syndromes. SeminOncol 1992;19:1925-1929.
10. Haase D Germing U Schanz J Pfeilstöcker M Nösslinger T Steidl N: New insights into the prognostic impact of the karyotype in MDS andcorrelation with subtypes: evidence from a core dataset of 2124 patients. Blood 2007 110: 4385-4395
11. De Witte T Hermans J Vossen J Bacigalupo A. Meloni G. Jacobsen N. et al:Haematopoietic stem cell transplantation for patients with myelodysplastic syndromes and secondary acute myeloid leukemias: A report on behalf of the Chronic Leukaemia Working Party of the European Group for Blood and Marrow Transplantation (EBMT).Br J Haematol2000;110:620-630.