UNIVERSIDADE FEDERAL DO CEARÁ Hospital Universitário Walter Cantídio
MYELODYSPLASTIC SYNDROMES DEMOGRAPHIC, CLINICAL FEATURES AND OUTCOME IN BRAZIL
Silvia Magalhães Universidade Federal do Ceará - Brazil 2016
MYELODYSPLASTIC SYNDROMES Fortaleza - Ce
Campinas - SP
BRAZIL median age at diagnosis: 58,5 y (15-89) 206 patients Magalhรฃes SMM et al Leuk Res 2001; 26: 525 Universidade Federal do Cearรก
MDS – BRAZILIAN REGISTRY
• 476 patients (2003 – 2007) • 12 Brazilian tertiary centers • Median age at time of diagnosis: 68.3 years • Hb at presentation was 8g/dL with no difference in severity of cytopenias between regions
• Predominant subtype: RCMD • Median time of follow-up was 31m Magalhães SMM et al Blood 2010; 116: abst 1884 Universidade Federal do Ceará
MDS – BRAZILIAN REGISTRY
OS 72m 56m 44m
Follow-up 23m 31m 22m
p=0,002
survival in different Brazilian regions Magalhães SMM et al Blood 2010; 116: abst 1884 Universidade Federal do Ceará
IS MYELODYSPLASTIC SYNDROME THE SAME EVERYWHERE?
GENETIC BACKGROUND ENVIRONMENTAL EXPOSURE Magalhรฃes SMM et al Leuk Lymphoma 2013; 54: 1564 Universidade Federal do Cearรก
Beyond race-related disparities - is myelodysplastic syndrome the same everywhere? Magalhรฃes SMM et al Leuk Lymphoma 2013; 54: 1564
SMD de novo
100
Age Median of age (y) (range) <60 years
67 (15-88) 38%
Number of cytopenias (IPSS) 0/1
55/100 (55%)
2/3
45/100 (45%)
MDS subtypes (WHO 2008) RCUD/RARS/Isolated del(5q)/RCMD RAEB I/RAEB II
89/100 (89%) 9/100 (9%)
IPSS Low/Int I
51/58 (88%)
Int II/High
7/58 (12%) Universidade Federal do Cearรก
IS MYELODYSPLASTIC SYNDROME THE SAME OVER TIME? 2003 - 2007 2008 - 2012 2013 - 2016 DEMOGRAPHIC TRANSITION ACCESS TO HEALTH FACILITIES Universidade Federal do Cearรก
Myelodysplastic syndromes in South America: A multinational study of 1080 patients
• Argentine: 635 (20 centers) • Brazil: 345 (2 University centers) • Chile: 100
retrospective primary MDS
Belli CB et al Am J Hematol 2015 Oct; 90: 851 Universidade Federal do Ceará
Universidade Federal do Cearรก
Universidade Federal do Cearรก
median age Argentine 7% Brazil 8% Chile 19%
69y 68y 64y
Distribution according to the range of age Universidade Federal do Cearรก
M/F ratio 0.8 <45y
Distribution according to age and gender Universidade Federal do Cearรก
BRAZIL
ARGENTINE
CHILE
RARS
10%
3%
1%
p<0.001
RCMD
35%
44%
53%
p=0.004
Distribution according to WHO categories
Universidade Federal do Cearรก
9.6 g/dL 8.8 g/dL
Hb < 7g/dL 10% 26% 24% AR BR CH
8.7 g/dL
Distribution according to hemoglobin level Universidade Federal do Cearรก
The frequency of abnormal karyotype was similar among the three series Most frequent cytogenetic abnormalities CHILE: higher frequency of complex karyotype BRAZIL: higher frequency of del(5q) ns
Universidade Federal do Cearรก
Distribution according to IPSS-R categories CHILE: higher frequency of VERY HIGH risk (Ch 10%
Ar 20% Br 13%)
Universidade Federal do Cearรก
TREATMENT HSCT: 4.9% AR and 6.4% BR
HMT: 17.5% AR and 7.5% BR
ICT: similar
No Chilean patient received HSCT, HMT or ICT HSCT: hematopoietic stem cell transplantation HMT: hypomethylating therapy ICT: iron chelation therapy Universidade Federal do Cearรก
OVERALL SURVIVAL
OS was lower in CHILE Median followup: 21m (1-266)
Universidade Federal do Cearรก
SUMMARY
• Chile: younger mean age and higher predominance of cases < 40 y
• Brazil and Chile: higher frequency of Hb < 7g/dL and higher percentage of pancytopenic patients
• CHILE: higher frequency of higher risk patients: more severe cytopenias, more complex karyotypes, shorter median survival
• Different public disease modifying therapy supply HMT)
Universidade Federal do Ceará
(HSCT,
QUESTIONS Is median age rising as registries are improving? Are there more difficulties to reach a specialized hematology center? Different patient referral patterns? Different public health care systems/ structure?
Universidade Federal do Cearรก
Carolina B Belli Yesica Bestach Irene B Larripa Roberta SS Tanizawa Graciela Alfonso Jacqueline Gonzalez
Mariana Rosenhain Nora Watman Marcela CA Silva Pedro N Aranguren Hernan G Rivello
Elvira RP Velloso (USP) Ronald F Pinheiro (UFC) Silvia M M MagalhĂŁes (UFC)
Maria S Undurraga Ximena Valadares
Universidade Federal do CearĂĄ
Steensma et al. BMC Cancer 2016; 16: 652. Universidade Federal do Cearรก
Steensma et al. BMC Cancer 2016; 16: 652. Universidade Federal do Cearรก
Steensma et al. BMC Cancer 2016; 16: 652. Universidade Federal do Cearรก
UNIVERSIDADE FEDERAL DO CEARÁ Hospital Universitário Walter Cantídio Serviço de Hematologia
silviamm@ufc.br