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
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