Registro de SMD Brasilero - Dra Silvia Magalhaes

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