YÜKSEK RİSKLİ MYELODİSPLASTİK SENDROM TANILI HASTALARIMIZIN RETROSPEKTİF DEĞERLENDİRİLMESİ
Giriş: Myelodisplastik sendrom (MDS) sitopenilerle seyreden, kemik iliğinin klonal bir hastalığıdır. Çeşitli skorlama sistemleri ile düşük ve yüksek riskli olarak sınıflandırılmıştır. Mortalite ve morbidite açısından tedaviye erken başlanması, takiplerin düzenli yapılması önemlidir. İzmir Bozyaka Sağlık Uygulama ve Araştırma Hastanesi hematoloji kliniğinde takipli 14 yüksek riskli MDS hastasını incelemeyi amaçladık. Gereç ve Yöntem: MDS nedeniyle takip edilen hastalardan yüksek riskli olanlar seçildi ve retrospektif olarak incelendi. Bulgular: Çalışmaya alınan 14 hastanın 6’sı kadın (%42,9), 8’i erkek (%57,1) olup medyan yaş 77 (65-81) idi. Hastaların yalnızca 1(%7,1)’inde anormal sitogenetik gözlenirken, 1 hastada 7q delesyonu (%7,1), 1 hastada 20q delesyonu (%7,1) gözlendi. 14 hastanın hepsine ilk sıra tedavi başlanmış olup 12 hasta (%85,7) azasitidin 2 2 75mg/m 7gün, 2 hasta (%14,3) desitabin tedavisi 20mg/m 5 gün aldığı izlendi. Hastalardan 2 (%13,3)’sinin demir şelasyon tedavisi aldığı, 12 (%86,7)’sinin şelasyon tedavisi almadığı görüldü. Tanı anı mean blast yüzdesi 12 iken, tedavi sonrası değerlendirmede mean blast yüzdesinin 14 (2-40)’e yükseldiği gözlendi. Hastaların %50sinde relaps görülürken diğer yarısında görülmedi. Hastaların hiçbirisine allojenik kemik iliği transplantasyonu yapılmadığı görüldü. Hastaların ortalama genel sağkalımları 23 ay (5-55) iken hastalıksız sağ kalım 14 ay (0-36) olarak saptandı. Sonuç: Hastalarımızın demografik verileri literatürle benzerdi. Genel sağkalım ve hastalıksız sağ kalım yüksek riskli hastalarda literatürle uyumluydu.
RETROSPECTIVE EVALUATION OF OUR PATIENTS WITH DIAGNOSIS HIGHER-RISK MYELODYSPLASTIC SYNDROMES
Introduction: Myelodysplastic syndrome (MDS) is a bone marrow clonal disease with cytopenias. It is classified as low and high risk with various scoring systems. Interms of mortality and morbidity, it is important that treatment is started early and that follow-ups are done regularly. We aimed to examine 14 high-risk MDS patients who were admitted at Department of General Haemotology, University of Health Science Izmir Bozyaka Education and Research Hospital Material and Methods: Patients who were followed up for MDS were selected for high-risk and retrospectively studied. Results: Of the 14 patients studied, 6 were female (42.9%), 8 were male (57.1%) and median age was 77 (65- 81) years. Only 1 (7.1%) of the patients had abnormal cytogenetic findings, 7q deletion was observed in 1 patient (7.1%) and 20q deletion in 1 patient (7.1%). 12 patients (85.7%) received azacitidine 75mg / m 2 for 7 days and 2 2 patients (14.3%) received decitabine therapy 20mg / m for 5 days. 2 (13.3%) patients received iron chelation therapy and 12 (86.7%) patients did not receive chelation therapy. The mean blast rate was 12 when the diagnosis was made, where as the mean blast rate increased to 14 (2-40) after the treatment. Relapse was seen in 50% of the patients but not in the other half. None of the patients had anyallogeneic bone marrow transplantation. The mean overall survival of the patients was 23 months (5-55), while disease-free survival was 14 months (0-36). Conclusion: The demographic data of our patients were similar to the literature. Overall survival and disease-free survival were in accordance with the literature in high-risk patients.
___
- Neukirchen J, Schoonen WM, Strupp C, Gattermann N, Aul C, Haas R et al. Incidence and prevalence of myelodysplastic
syndromes: data from the Dusseldorf MDS-registry. Leuk Res 2011; 35(12): 1591-6.
- Türk hematoloji derneği myelodiplastik sendrom kılavuzu 2015.
- Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau et al. The 2016 revision to the World Health Organization
classification of myeloid neoplasms and acute leukemia. Blood 2016; 127(20): 2391-405.
- Garcia-Manero G, Shan J, Faderl S, Cortes J, Ravandi F, Borthakur G et al. A prognostic score for patients with lower risk
myelodysplastic syndrome. Leukemia 2008; 22(3): 538-43.
- Malcovati L, Germing U, Kuendgen A, Della Porta MG, Pascutto C, Invernizzi Ret al. Time-dependent prognostic scoring
system for predicting survival and leukemic evolution in myelodysplastic syndromes. J Clin Oncol 2007; 25(23): 3503-10.
- Greenberg PL, Tuechler H, Schanz J, Sanz G, Garcia-Manero G, Sole F et al. Revised ınternational prognostic scoring
systemfor myelodysplastic syndromes. Blood 2012; 120(12): 2454-65.
- Della Porta MG, Malcovati L, Boveri E, Travaglino E, Pietra D, Pascutto C et al. Clinical relevance of bone marrow fibrosis
and CD34-positive cell clusters in 41 primary myelodysplastic syndromes. J Clin Oncol 2009; 27(5): 754-62.
- Germing U, Hildebrandt B, Pfeilstocker M, Nosslinger T, Valent P, Fonatsch C et al. Refinement of the international
prognostic scoring system (IPSS) by including LDH as an additional prognostic variable to improve risk assessment in
patients with primary myelodysplastic syndromes (MDS). Leukemia 2005; 19(12): 2223-31.
- Thol F, Friesen I, Damm F, Yun H, Weissinger EM, Krauter J et al. Prognostic significance of ASXL1 mutations in patients
with myelodysplastic syndromes. J Clin Oncol 2011; 29(18): 2499-506.
- DeSantis CE, Lin CC, Mariotto AB, Siegel RL, Stein KD, Kramer JL et al. Cancer treatment and survivorship statistics
2014. CA Cancer J Clin 2014; 64(4): 252-71.
- Jansen AJ, Essink-Bot ML, Beckers EA, Hop WC, Schipperus MR, Van Rhenen DJ. Quality of life measurement in patients
with transfusion dependent myelodysplastic syndromes. Br J Haematol 2003; 121(2): 270-4.
- Jonas BA, Greenberg PL. MDS prognostic scoring systems – past, present, and future. Best Pract Res Clin Haematol
2015; 28(1): 3-13.
- Hadman M, Deeg HJ. Hematopoietic cell transplantation for older patients with MDS. Mediterr J Hematol Infect Dis 2014;
6(1): e2014056.
- Malcovati L, Porta MG, Pascutto C. Prognostic factors and life expectancy in myelodysplastic syndromes classified
according to WHO criteria: a basis for clinical decision making. J Clin Oncol 2005; 23(30): 7594-603.