2022, Number 2
<< Back Next >>
Med Int Mex 2022; 38 (2)
Myelodysplastic syndromes: an update for the non-hematologist
Hernández-Sómerson MA, Huertas-Rodríguez G, Medina-Lee LD, Ortiz-Arroyo J, Pabón-Barrera J, Escobar-Sáenz JA
Language: Spanish
References: 39
Page: 366-377
PDF size: 487.89 Kb.
ABSTRACT
Myelodysplastic syndromes comprise a heterogeneous group of neoplasms that affect
hematopoietic stem cells, which are related to an increased risk of developing acute
myeloid leukemia. Its clinical presentation is highly variable and should be suspected
in the presence of persistent cytopenias without clear cause. The cornerstone of the
diagnosis is bone marrow biopsy, where dysplastic changes in one or more cell lines,
the presence of ringed sideroblasts, the number of blasts and the cytogenetic study
make it possible to classify this disease in six different subtypes. Currently, several
scores are available to estimate the prognosis leading to an appropriate choice of
treatment based on survival and the risk of developing acute myeloid leukemia. This
work updates the available literature regarding diagnosis, classification, prognosis
systems and treatment of the myelodysplastic syndromes with the objective of providing
the non-hematologist with tools that allow early detection and directing the
hematologist to a timely treatment.
REFERENCES
Valent P, Horny HP, Bennett JM, Fonatsch C, Germing U, Greenberg P, et al. Definitions and standards in the diagnosis and treatment of the myelodysplastic syndromes: Consensus statements and report from a working conference. Leukemia Research 2007; 31 (6): 727-736. https:// doi.org/10.1016/j.leukres.2006.11.009.
Tefferi A, Vardiman JW. Myelodysplastic syndromes. N Engl J Med 2009; 361 (19): 1872-1885. DOI: 10.1056/ NEJMra0902908.
Greenberg PL, Stone RM, Al-Kali A, Barta SK, Bejar R, Bennett JM, et al. Myelodysplastic syndromes, version 2.2017. Clinical practice guidelines in oncology. J Natl Compr Canc Netw 2017; 15 (1): 60-87. doi: 10.6004/jnccn.2017.0007.
Strom SS, Velez-Bravo V, Estey EH. Epidemiology of myelodysplastic syndromes. Semin Hematol 2008; 45 (1): 8-13. doi: 10.1053/j.seminhematol.2007.10.003.
Ma X. Epidemiology of myelodysplastic syndromes. Am J Med 2012; 25 (7 Suppl): S2-5. doi: 10.1016/j.amjmed. 2012.04.014.
Sekeres MA. The epidemiology of myelodysplastic syndromes. Hematol Oncol Clin North Am 2010; 24 (2): 287-94. doi: 10.1016/j.hoc.2010.02.011.
Shallis RM, Ahmad R, Zeidan AM. The genetic and molecular pathogenesis of myelodysplastic syndromes. Eur J Haematol 2018; 101 (3): 260-271. doi: 10.1111/ejh.13092.
Papaemmanuil E, Gerstung M, Malcovati L, Tauro S, Gundem G, Van Loo P, et al. Clinical and biological implications of driver mutations in myelodysplastic syndromes. Blood 2013; 122: 3616-27. https://doi.org/10.1182/ blood-2013-08-518886.
Adès L, Itzykson R, Fenaux P. Myelodysplastic syndromes. Lancet 2014; , 383 (9936): 2239-2252. doi: 10.1016/S0140- 6736(13)61901-7.
Nisse C, Haguenoer JM, Grandbastien B, Preudhomme C, et al. Occupational and environmental risk factors of the myelodysplastic syndromes in the North of France. Br J Haematol 2001; 112: 927-35. doi: 10.1046/j.1365- 2141.2001.02645.x.
Du Y, Fryzek J, Sekeres MA, Taioli E. Smoking and alcohol intake as risk factors for myelodysplastic syndromes (MDS). Leuk Res 2010; 34 (1): 1-5. doi: 10.1016/j.leukres. 2009.08.006.
Foran JM, Shammo JM. Clinical presentation, diagnosis and prognosis of myelodysplastica syndromes. Am J Med 2012; 125 (7suppl): s6-13. doi: 10.1016/j.amjmed.2012.04.015.
Garcia-Manero G. Myelodisplastic syndromes: 2015 update on diagnosis, risk-stratification and management. Am J Hematol 2015; 90 (9): 832-841. doi: 10.1002/ajh.24102.
Gangat N, Patnaik MM, Tefferi A. Myelodysplastic syndromes: Contemporary review and how we treat. Am J Hematol 2015; 91 (1): 76-89. doi: 10.1002/ajh.24253.
Steensma Dp, Bennett JM. The myelodysplastic syndromes: diagnosis and treatment. Mayo Clin Proc 2006; 81 (1): 104- 30. doi: 10.4065/81.1.104.
Nguyen PL. The myelodysplastic syndromes. Hematol Oncol Clin North Am 2009; 23 (4): 675-91. doi: 10.1016/j. hoc.2009.04.008.
Wang SA. Diagnostic of myelodysplastic syndromes in cytopenic patients. Hematol Oncol Clin North Am 2011; 25 (5): 1085-110. doi: 10.1016/j.hoc.2011.09.009.
Zini G. Diagnostics and prognostication of myelodysplastic syndromes. Ann Lab Med 2017; 37 (6): 465-74. doi: 10.3343/alm.2017.37.6.465.
Verburgh E, Achten R, Maes B, Hagemeijer A, Boogaerts M, De Wolf-peeters C, et al. Additional prognostic value of bone marrow histology in patients subclassified according to International Prognostic Scoring System for myelodysplastic syndromes J Clin Oncol 2003; 21(2): 273-82. doi: 10.1200/JCO.2003.04.182.
Mufti GJ, Bennett JM, Goasgen J, Bain BJ, Baumann I, Brunning R, et al. Diagnostic and classification of myelodysplastic syndromes: International Working Group on morphology of myelodysplastic syndrome (IWGM-MDS) consensus proposals for the definition and enumeration of myeloblasts and ring sideroblasts. Haematologica 2008; 93(11): 1712-7. doi: 10.3324/haematol.13405.
Platzbecker U, Santini V, Mufti GJ, Haferlach C, Maciejewski JP, Park S, et al. UpdAte on developments in the diagnosis and prognostic evaluation in patients with myelodysplatic sybdromes (MDS): consensus statements and report from an expert workshop. Leuk Res 2012; 36(3): 64-70. https:// doi.org/10.1016/j.leukres.2011.11.005.
Haferlach T. Molecular genetics in myelodysplastic syndromes. Leuk Res 2012; 36 (12): 1459-1462. doi: 10.1016/j. leukres.2012.08.009.
Haase D. Cytogenetics features in myelodysplastic syndromes. Ann Hematol 2008; 87(7): S15-S26. doi: 10.1007/ s00277-008-0483-y.
Schanz J, Tuchler H, Sole F, Mallo M, et al. New comprehensive cytogenetic scoring system for primary myelodysplastic syndromes (MDS) and oligoblastic acute myeloid leukemia after MDS derived from an international databas emerge. J Clin Oncol 2012; 30 (8): 820-829. doi: 10.1200/ JCO.2011.35.6394.
Papaemmanuil E, Gerstrung M, Malcovati, Tauro S, Gundem G, Van Loo P, et al. Clinical and biological implications of driver mutations in myelodysplastic syndromes. Blood 2013; 122 (22): 3616-3627. https://doi.org/10.1182/ blood-2013-08-518886.
Montalban-Bravo G, Garcia-Manero G. Myelodysplastic syndromes: 2018 update on diagnosis, risk-stratification and management. Am J Hematol 2018; 93: 129-147. doi: 10.1002/ajh.24930.
Bennett JM, Catovski D, Daniel MT, Flandrin G, Galton DA, Gratnick HR, et al. Proposals for the classification of the acute leukaemias, French- American-British (FAB) cooperative group. Br J Haematol 1976; 33 (4): 451-8. doi: 10.1111/j.1365-2141.1976.tb03563.x.
Bennett JM, Catovski D, Daniel MT, Flandrin G, Galton DA, Gratnick HR, et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 1982; 51 (2): 189-99.
Vardiman J. The classification of MDS: from FAB to WHO and beyond. Leuk Res 2012; 36 (12): 1453-8. doi: 10.1016/j. leukres.2012.08.008.
Bennett JM. World Health organization classification on the leukemias and myelodysplastic syndrome. Int J Hematol 2000; 72 (2): 131-3.
Vardiman J, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood 2009; 114 (5): 937-51. doi: 10.1182/ blood-2009-03-209262.
Arber DA, Orazi A, Hassenian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision of World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127 (20): 2391-405. https://doi. org/10.1182/blood-2016-03-643544.
Greenberg P, Cox C, Lebeau MM, Fenaux P, Morel P, Saz G, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997; 89 (6): 2079-88.
Cazzola M, Malcovati L. Prognostic classification and risk assessment in myelodysplastic syndromes. Hematol Oncol Clin North Am 2010; 24 (2): 459-68. doi: 10.1016/j. hoc.2010.02.005.
Nazha A, Sekeres MA. Improving prognostic modeling in myelodysplastic syndromes. Clin Hematol Malig Rep 2016; 11 (6): 395-401. doi: 10.1007/s11899-016-0342-1.
Malcovati L, Germing U, Kuendgen A, Della Porta MG, Pascutto C, Giagounidis A, et al. Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. J Clin Oncol 2007; 25 (23): 3503-10. doi: 10.1200/JCO.2006.08.5696.
Greenberg PL, Tuechler H, Schanz J, Sanz G, García-Manero G, Solé F. Revised international prognostic scoring system for myelodysplastic syndromes. Blood 2012; 120 (12): 2054-2065. doi: 10.1182/blood-2012-03-420489.
Santini V. Treatment of low-risk myelodysplastic syndromes. Hematology Am Soc Hematol Educ Program 2016; 2016 (1): 462-469. https://dx.doi.org/10.1182%2Fashed ucation-2016.1.462.
Komrokji R. Current state of the art: Management of higher risk myelodysplastic syndromes. Clin Lymphoma Myeloma Leuk 2016; 16: S39-43. doi: 10.1016/j.clml.2016.02.019.