Pb2015: diagnosis, classification and stratification of myelodysplastic syndromes in algeria

Topic: 10. Myelodysplastic syndromes - Clinical Background: The diagnosis of myelodysplastic syndromes (MDS) is based on cytological and cytogenetic data Aims: Our objective is the application of WHO recommendations for the diagnosis of MDS in order to improve their therapeutic management Methods: This is a retrospective study of 638 MDS from 10 haematology departments. We analysed the haemogram, cytology, karyotype and FISH targeting 5q, 7q, 20q,17p13(P53); 575 met the WHO recommendations for diagnosis Results: This is about 268 M/307W;sex ratio 0.87;median age= 66 years;Hg: 8< Hb <10g/dl =27.3%,Hb ≤ 8g/dl=51%,Macrocytic =30.7%;neutropenia ≤800/µl =18%, thrombocytopenia < 50000/µl = 23.3%,50000/µl ≤ plaq <100000/µl =21.2%;thrombocytosis =2.6%;bicytopenia =30.4%;pancytopenia =25.5%;Blood smear: macrocytosis =73.7%,pseudopelgers=25.2%,hypogranulated=46%, macrothrombocytes =48.5%;blasts< 5% =93.3% ≥5 < 20% = 6.7%,Myelogram: dysemegacarypoiesis= 47.4%,dysgranulopoiesis =53%, dyserythropoiesis =89.3%,multilineage dysplasia=43.4%;bone marrow blasts,> 2< 5% =13.7%,≥ 5% ≤10% =16.6%,< 20% ˃10% =11%,Sideroblastic ring in crown >15% =19.6%,BOM done =17.7%,. CMF done in 4.8%.Cytogenetics:185karyotypes done(32%),normal in 62%,pathological in 38%. FISH done in 141pts,karyotype coupled with FISH were pathological in 65/185 pts(35%):one abnormality in 49,2%,two abnormalities in17%,complex to 3 in 9,2% and ˃3 in 24,6%: del (5q)/-5 in 46%,isolated in 21.5%,del 7q/-7 in 16.9%;del 17p13(p53) in7.6%,tri 8 in13.8%,del 20q in 13.8%,del11q in7.6%,duplications in 9.2%,monosomies in 24.6%,translocations in17%,and other anomalies in 20%. WHO/RA =24%,RN= 0.86%,RT=3.3%,RCDML= 29.2%,RCUDS =8.8%,RCMDSI =2.2%,RAEB1=14%,RAEB2=5%,MDS-i =2 pts, Sd 5q- = 13/185pts (7%);Cytogenetics: very good =1.2%,good =76.3%,intermediate =8.4%,poor= 6.6%,very poor=7.2%;IPSS score on165pts: Low =32%,Intermediate1=47.2%,Intermediate 2=15.7%,High =4.8%;IPSS-R: Very Low =5.4%,Low= 36.3%,Intermediate =32%,High =10.3%,Very High =15.7%.WPSS:very low =24.2%,low=24.8%,intermediate =17%;high =26%;very high =7.8%.Become:277 died (48%),38.2% by transformation,14.4% by comorbidities,83.7% of the high risk,31.8% of the low and 45.2% of the intermediate;298 are alive (52%),median OS and PFS of 39 months,96 months for the low,34months for the intermediate,13.6months for the high and the very high Summary/Conclusion: The diagnostic recommendations were applied in all the patients of the study on the cytological level and only in 32% on the cytogenetic level, the frequency of cytogenetic abnormalities found was 35%, we classified the100% on the cytology which was decisive, the survival was influenced by the type of MDS, the number of cytopenias, by unfavourable cytogenetics, the high prognostic risks of which 88% death. Hence, the interest in developing the missing tools for the diagnosis of MDS in order to improve therapeutic management in the future Keywords: Cytogenetic abnormalities, Myelodysplastic syndrome, WHO classification