Scientific Online Resource System

Scripta Scientifica Medica

Novel approaches in the classification and risk assessment of patients with myelodysplastic syndromes-clinical implication

Ilina Micheva, Rosen Rachev, Hinco Varbanov, Vladimir Gerov, Liana Gercheva

Abstract

INTRODUCTION: New prognostic systems have been proposed aimed at improving the ability to predict survival and progression in myelodysplastic syndome (MDS) patients, as well as to select the accurate therapy.

PURPOSE: The aim of this study was to determine the prognostic score for patients with MDS, diagnosed in the Hematology Clinic, University hospital, Varna, comparing different prognostic scoring systems.

MATERIALS AND METHODS: 92 patients with MDS, diagnosed between 2004 and 2012 were included in the study. The mediana of age was 72 (20-91 years), 87% >60 years of age, 52 men and 41 women. The prognostic score was determined according to IPSS, WPSS, MDACSS. The parameters assessed were WHO type, karyotype, cytopenias, percentage of marrow blasts, age, performing status and transfusion dependence.

RESULTS: Cytogenetic studies were performed in 100% of patients. Abnormalities were found In 34.8%, the most common-del-5q (14.6%), -Y (4.5%), complex karyotype (8%), +8 (2.2%). Patients were distributed according to the cytogenetic risk -low-87.2%, intermediate-7.7%, high-5.1%. According to IPSS 41% of the patients were with low risk, 34%-Int-1, 16%-Int-2, high-9%.  WPSS distributed the patients in 5 groups: very low-11%, low risk-43%, int-21%, high-20%, very high-5%. According to MDACSS 14% of the patients were with low, 54%-Int-1, 18%-Int2, 13% with high risk.

CONCLUSION: Significant correlation was found between the risk groups and AML transformation and survival. However, the comparison between the different scoring systems demonstrated the advantage of prognostic systems, based on broader range of clinical parameters in better distribution of intermediate risk patients and the precise determination of high and very high risk. 


Keywords

myelodysplastic syndrome; classification; prognostic systems

Full Text


References

Bejar R, Levine R, Ebert BL. Unraveling the molecular pathophysiology of myelodysplastic syndromes. J Clin Oncol. 29, 2011, 5, 504-15.

Bejar R, Stevenson K, Abdel-Wahab O, Galili N, Nilsson B, Garcia-Manero G, et al. Clinical effect of point mutations in myelodysplastic syndromes. N Engl J Med. 364, 2011, 26, 2496-506.

Bennett JM. A comparative review of classification systems in myelodysplastic

syndromes (MDS). Semin Oncol. 32, 2005, 4, Suppl 5, S3-10

Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G, et al. International

scoring system for evaluating prognosis in myelodysplastic syndromes. Blood. 89, 1997, 6, 2079-2088.

Greenberg PL, Tuechler H, Schanz J, Sanz G, Garcia-Manero G, Solé F, Bennett JM, et al. Revised international prognostic scoring system for myelodysplastic syndromes. Blood. 120, 2012,12, 2454-65.

Haase D, Germing U, Schanz J, Pfeilstöcker M, Nösslinger T, Hildebrandt B, et al. New insights into the prognostic impact of the karyotype in MDS and correlation with subtypes: evidence from a core dataset of 2124 patients. Blood. 110, 2007, 13, 4385-95.

Kantarjian H, O'Brien S, Ravandi F, Cortes J, Shan J, Bennett JM, et al. Proposal for a new risk model in myelodysplastic syndrome that accounts for events not considered in the original International Prognostic Scoring System. Cancer.

, 2008, 6, 1351-61.

Malcovati L, Germing U, Kuendgen A, Della Porta MG, Pascutto C, Invernizzi R, et al. Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. J Clin Oncol. 25, 2007, 23, 3503-3510.

Schanz J, Tüchler H, Solé F, Mallo M, Luño E, Cervera J, et al. New comprehensive cytogenetic scoring system for primary myelodysplastic syndromes (MDS) and oligoblastic acute myeloid leukemia after MDS derived from an international database merge. J Clin Oncol. 30, 2012, 8, 820-829.

Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, Fourth Edition EDS. WHO, 2008.

Walter MJ, Ding L, Shen D, Shao J, Grillot M, McLellan M, et al. Recurrent DNMT3A mutations in patients with myelodysplastic syndromes. Leukemia. 25, 2011, 7, 1153-8.




DOI: http://dx.doi.org/10.14748/ssm.v46i1.647
Array
Article Tools
Email this article (Login required)
About The Authors

Ilina Micheva
Medical University of Varna
Bulgaria

Hematology Division, Department of Internal Medicine

Rosen Rachev
Medical University of Varna
Bulgaria

Hematology Division, Department of Internal Medicine

Hinco Varbanov
Medical University of Varna
Bulgaria

Hematology Division, Department of Internal Medicine

Vladimir Gerov
MBAL St. Marina
Bulgaria

Clinic of Hematology

Liana Gercheva
Medical University of Varna
Bulgaria

Hematology Division, Department of Internal Medicine

Font Size


|