The Myelodysplastic Syndrome in Northern and Central Moravia Project
Authors:
D. Starostka 1; P. Rohoň 2; D. Adamová 3; E. Kadlčková 4; M. Brejcha 5; D. Janek 6; R. Jochymek 7; M. Švarná 8; N. Rytiková 9; J. Laská 10; K. Vinklárková 11; M. Holzerová 2; M. Tichý 12
Authors‘ workplace:
Oddělení klinické hematologie, Nemocnice s poliklinikou Havířov, p. o.
1; Hemato-onkologická klinika, Fakultní nemocnice Olomouc
2; Hematologicko-transfuzní oddělení, Slezská nemocnice v Opavě, p. o.
3; Hematologicko-transfuzní oddělení, Krajská nemocnice T. Bati Zlín, a. s.
4; Hematologické oddělení, Nemocnice Nový Jičín, a. s.
5; Hematologicko-transfuzní oddělení, Nemocnice s poliklinikou Karviná-Ráj, p. o.
6; Hematologicko-transfuzní oddělení, Nemocnice Třinec, p. o.
7; Hematologicko-transfuzní oddělení, Nemocnice ve Frýdku-Místku, p. o.
8; Hematologická ambulance, Sdružené zdravotnické zařízení Krnov, p. o.
9; Oddělení klinické hematologie, Městská nemocnice Ostrava, p. o.
10; Hematologicko-transfúzní oddělení, Nemocnice Hranice, a. s.
11; Ústav klinické a molekulární patologie, Lékařská fakulta UP v Olomouci
12
Published in:
Transfuze Hematol. dnes,21, 2015, No. 2, p. 92-100.
Category:
Comprehensive Reports, Original Papers, Case Reports
Overview
Introduction:
The primary aim of the project was to collect epidemiologic data concerning MDS and its therapy in Northern and Central Moravia. The secondary aim was to improve the diagnostics and prognostic stratification of MDS.
Methods:
The data of newly diagnosed MDS and MDS/MPN cases were collected in the region with a population of 2.2 million inhabitants over 1 year. The data included: sex, age at the time of diagnosis, diagnostic category according to the WHO classification, primary and secondary MDS, IPSS, WHO performance status, comorbidity, treatment.
Results:
107 new cases of MDS and MDS/MPN were diagnosed in the area. The incidence of MDS and MDS/MPN was 4.9 per 100 000 inhabitants. The median age was 70 years (min. 43 – max. 84). The incidence of diagnostic categories was: RCUD 15.0%, RARS 9.3%, RCMD 37.4%, RAEB-1 15%, RAEB-2 10.3%, 5q- syndrome 0.09%, MDS-U 0.09%, CMML-1 4.7%, CMML-2 4.7%, MDS/MPS-U 1.9%. The diagnostic concordance of primary and secondary cytological analysis was 92%. The diagnostic concordance of cytological and histological analysis was 74%. The distribution of the IPSS categories was: low risk 32.9%, intermediate-1 risk 44.7%, intermediate-2 risk 16.4%, high-risk 6.0%. The distribution of PS scores: score 0 – 24%, score 1 – 50%, score 2 – 11%, score 3 – 15%. Primary MDS was diagnosed in 84.1%, secondary MDS in 15.9% of patients. Significant comorbidities were reported in 72% of patients: cardiovascular 52.3%, endocrinological including diabetes mellitus 27%, gastrointestinal 12.1%, significant chronic kidney disease 5.6%, rheumatological 5.6%, pulmonary 4.7%, neurological and psychiatric 7.5%. A second neoplasm was diagnosed in 18.7% of patients, 1 case of neoplastic triplicity was observed. Therapy included: watch and wait in 42%, erythrocyte transfusion in 50.5%, erythropoietin in 17.8%, chemotherapy in 12.1%, epigenetic therapy in 7.4 %, chelation in 4.7%, corticosteroids in 3.7%, allo-BMT in 1.9% patients.
Conclusions:
Basic demographic, diagnostic, prognostic and therapeutic data concerning MDS and MDS/MPN were collected in the area of Northern and Central Moravia. Given patient age and comorbidities, the therapeutic options in MDS and MDS/MPN remain limited. High-risk patients should always be managed in transplantation centres.
Key words:
MDS, MDS/MPN, diagnosis, therapy, comorbidity
Sources
1. Neukirchen J, Schoonen WM, Strupp C, et al. Incidence and prevalence of myelodysplastic syndromes: data from the Dusseldorf MDS – registry. Leuk Res 2011;35: 1591–1596.
2. Beris P, Georgiou G. Overview of myelodysplastic syndromes. Semin Hematol 2012; 49: 287–294.
3. Garcia-Manero G. Myelodysplastic syndromes: 2012 update on diagnosis, risk-stratification, and management. Am J Hematol 2012; 87: 692–701.
4. Greenberg P, Cox C, LeBeau MM, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997; 89: 2079–2088.
5. Greenberg PL, Tuechler H, Schanz J, et al. Revised international prognostic scoring system for myelodysplastic syndromes. Blood 2012; 120: 2454–2465.
6. Breccia M, Molica M, Zacheo I, Alimena G. Azacitidine for myelodysplastic patients aged > 65 years: a review of clinical efficacy. Expert Opin Pharmacother 2014;15(11): 1621–1630.
7. Gyurkocza B, Deeg HJ. Allogeneic hematopoietic cell transplantation for MDS: for whom, when and how? Blood Rev 2012; 26: 247–254.
8. Della Porta MG, Alessandrino EP, Bacigalupo A, et al. Predictive factors for the outcome of allogeneic transplantation in patients with MDS stratified according to the revised IPSS-R. Blood 2014; 123: 2333–2342.
9. Fenaux P, Ades L. How we treat lower-risk myelodysplastic syndromes. Blood 2013; 23: 4280–4286.
10. Ma X. Epidemiology of myelodysplastic syndromes. Am J Med 2012; 125: S2–5.
11. Rollison DE, Howlader N, Smith MT, et al. Epidemiology of myelodysplastic syndromes and chronic myeloproliferative disorders in the United States, 2001-2004, using data from the NAACCR and SEER programs. Blood 2008; 112: 45–52.
12. Cogle CR, Craig BM, Rollison DE, List AF. Incidence of the myelodysplastic syndromes using a novel claims-based algorithm: high number of uncaptured cases by cancer registries. Blood 2011; 117: 7121–7125.
13. McQuilten ZK, Wood EM, Polizzotto MN, et al. Underestimation of myelodysplastic syndrome incidence by cancer registries. Cancer 2014; 120 (1): 1686–1693.
14. Visser O, Trama A, Maynadie M, et al. Incidence, survival and prevalence of myeloid malignancies in Europe. Eur J Cancer 2012; 48: 3257–3266.
15. Germing U, Aul C, Niemeyer CM, et al. Epidemiology, classification and prognosis of adults and children with myelodysplastic syndromes. Ann Hematol 2008; 87: 691–699.
16. Della Porta MG, Malcovati L. Clinical relevance of extra-hematologic comorbidity in the management of patients with myelodysplastic syndrome. Haematologica 2009; 94: 602–606.
17. Naqvi K, Garcia-Manero G, Sardesai S, et al. Association of comorbidities with overall survival in myelodysplastic syndrome: development of a prognostic model. J Clin Oncol 2011; 29: 2240–2246.
18. Piccirillo JF, Tierney RM, Costas I, Grove L, Spitznagel EL Jr. Progno-stic importance of comorbidity in a hospital-based cancer registry. JAMA 2004; 291: 2441–2447.
19. Malcovati L, DellaPorta MG, Pascutto C, et al. Prognostic factors and life expectancy in myelodysplastic syndromes classified according to WHO criteria: a basis for clinical decision making. J Clin Oncol 2005; 23: 7594–7603.
20. Kuendgen A, Strupp C, Aivado M, et al. Myelodysplastic syndromes in patients younger than age 50. J Clin Oncol 2006; 24: 5358–5365.
21. Malcovati L, Della Porta MG, Cazzola M. Predicting survival and leukemic evolution in patients with myelodys-plastic syndrome. Haematologica 2006; 91: 1588–1590.
22. Sorror ML, Maris MB, Storb R, et al. Hematopoietic cell transplanta-tion (HCT)-specific comorbidity index: a new tool for risk assessment before allogeneic HCT. Blood 2005; 106: 2912–2919.
23. Dinmohamed AG, Visser O, van Norden Y, et al. Trends in incidence, initial treatment and survival of myelodysplastic syndromes: A population-based study of 5144 patients diagnosed in the Netherlands from 2001 to 2010. Eur J Cancer 2014; 50(5): 1004–1012.
24. Douvali E, Papoutselis M, Vassilakopoulos TP, et al. Safety and efficacy of 5-azacytidine treatment in myelodysplastic syndrome patients with moderate and mild renal impairment. Leuk Res 2013; 37(8): 889–893.
25. Malcovati L, Hellström-Lindberg E, Bowen D, et al. Diagnosis and treatment of primary myelodysplastic syndromes in adults: recommendations from the European LeukemiaNet. Blood 2013; 122(17): 2943–2964.
Labels
Haematology Internal medicine Clinical oncologyArticle was published in
Transfusion and Haematology Today
2015 Issue 2
Most read in this issue
- Von Willebrand disease
- Advancements in the treatment of adult acute lymphoblastic leukaemia
- Evaluation of bone marrow therapeutic response in patients with high risk myelodysplastic syndrome
- Monoclonal antibodies in the treatment of multiple myeloma