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Abstract
Myelodysplastic syndromes are a heterogeneous group of clonal hematological diseases which diagnosis requires a multidisciplinary scenario. In addition to clinical manifestations and bone marrow and peripheral blood features, karyotyping is mandatory for the diagnosis. The histopathological analysis of the bone marrow and the immunophenotyping by flow cytometry contribute to the diagnostic confirmation. The prognostic systems have been designed in order to apply therapeutic strategies adapted to risk. The aim of this work is to evaluate results of a survey designed to report on currently available diagnostic and therapeutic tools for hematologists in Argentina. The survey was fulfilled by 195 hematologists belonging to every Regional of the Argentine Society of Hematology, throughout the XXII Argentine Congress of Hematology. Results showed that complementary studies for the diagnosis are requested by physicians, despite differences either on the availability for diagnostic tests between Regionals or the type of attention. The most commonly used risk stratification systems are IPSS and IPSS-R. Main factors which are taken into consideration by the physician to decide the treatment differ between adult and pediatric hematologists. Support measures, erythropoietin and azacitidine are most frequently used for treatment of adults, whereas the indication for allogeneic stem cell transplantation is more common among pediatricians. Treatment discontinuation is mainly due to lack of response, with the exception of chelation therapy which also presents a similar proportion of adverse effects. Addressing the Argentinean reality will allow us to design collaborative strategies to achieve adequate access and equity to our patients.
References
2. Jaffe ES, Harris NL, Stein H, Vadiman JW (Eds). World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Haematopoietic and Lymphoid Tissues,
IARC Press: Lyon 2001.
3. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press:
Lyon 2008.
4. Tefferi A, Vardiman JW. Myelodysplastic syndromes. N Engl J Med. 2009; 361: 1872–1885. 5. Garcia-Manero G. Myelodysplastic syndromes: 2015 Update on diagnosis, risk-stratification and
management. Am J Hematol. 2015; 90: 831-841.
6. Arber DA, Orazi A, Hasserjian R y col. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016; 127:2391-2405.
7. Valent P, Horny HP, Bennett J y col. Definitions and standards in the diagnosis and treatment of the myelodysplastic syndromes: Consensus statements and report from a working conference.
Leuk Res. 2007; 31: 727-736.
8. Garcia-Manero G. Myelodysplastic syndromes: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol. 2014; 89: 97-108.
9. Sociedad Argentina de Hematología. Guias de Diagnostico y Tratamiento. www.sah.org.ar
10. Grupo Español de Síndromes Mielodisplásicos (GESMD). Sociedad Española de Hematología y Hemoterapia (SEHH). Guías españolas de diagnóstico y tratamiento de los síndromes
mielodisplásicos y la leucemia mielomonocítica crónica. Haematologica/edición española. 2012; 97 (Supl. 5).
11. Greenberg P, Cox C, LeBeau M y col. International International Scoring System for evaluating prognosis in myelodysplastic syndromes. Blood. 1997; 89: 2079-2088.
12. Greenberg PL, Tuechler H, Schanz J y col. Revised international prognostic scoring system for myelodysplastic syndromes. Blood. 2012; 120: 2454-2465.
13. Malcovati L, Germing U, Kuendgen A y col. Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. J ClinOncol. 2007;
25: 3503-10.
14. Malcovati L, Della Porta MG, Strupp C y col. Impact of the degree of anemia on the outcome of patients with myelodysplastic syndrome and its integration into the WHO classification-based Prognostic Scoring System (WPSS). Haematologica. 2011; 96: 1433-1440.
15. Kantarjian H, O’Brien S, Ravandi F y col. Proposal for a New Risk Model in Myelodysplastic Syndrome that accounts for events not considered in the original International Prognostic Scoring System. Cancer. 2008; 113: 1351-1361.
16. INDEC. Instituto Nacional de Estadísticas y Censos. Censo Nacional de Población, Hogares y Viviendas 2010. Censo Del Bicentenario Resultados Definitivos. Serie B Nº 2. Talleres
Gráficos Prix, Buenos Aires 2012.
17. De Roos AJ, Deeg HJ, Onstad L y col. Incidence of myelodysplastic syndromes within a nonprofit healthcare system in western Washington state, 2005-2006. Am J Hematol. 2010; 85:765-770.
18. Flores G, Basquiera A, Kornblihtt L y col. Datos epidemiológicos y pronósticos del Registro Argentino de Síndromes Mielodisplásicos (SMD). Hematología. 2016; 20: 27 – 34.
19. Senent L, Arenillas L, Luño E, Ruiz JC, Sanz G, Florensa L. Reproducibility of the World Health Organization 2008 criteria for myelodysplastic syndromes. Haematologica. 2013; 98: 568-575.
20. Font P, Loscertales J, Soto C y col. Interobserver variance in myelodysplastic syndromes with less than 5 % bone marrow blasts: unilineage vs. multilineage dysplasia and reproducibility
of the threshold of 2 % blasts. Ann Hematol. 2015; 94: 565-573.
21. Parmentier S, Schetelig J, Lorenz K y col. Assessment of dysplastic hematopoiesis: lessons from healthy bone marrow donors. Haematologica. 2012; 97: 723-730.
22. Della Porta MG, Malcovati L, Boveri E y col. Clinical relevance of bone marrow fibrosis and CD34-positive cell clusters in primary myelodysplastic syndromes. J ClinOncol. 2009;
27:754-762.
23. Porwit A, van de Loosdrecht AA, Bettelheim P y col. Revisiting guidelines for integration of flow cytometry results in the WHO classification of myelodysplastic syndromes-proposal from the
International/European Leukemia Net Working Group for Flow Cytometry in MDS. Leukemia. 2014; 28:1793-1798.
24. Bejar R, Stevenson K, Abdel-Wahab O y col. Clinical Effect of Point Mutations in Myelodysplastic Syndromes. N Engl J Med. 2011; 364:2496-2506.
25. Papaemmanuil E, Gerstung M, Malcovati L y col. Chronic Myeloid Disorders Working Group of the International Cancer Genome Consortium. Clinical and biological implications of driver mutations in myelodysplastic syndromes. Blood. 2013; 122: 3616-3627.
26. Steensma D, Bejar R, Jaiswal S y col. Clonal hematopoiesis of indeterminate potential and its distinction from myelodysplastic syndromes. Blood. 2015;126 :9-16.
27. Al Assaf C, Van Obbergh F, Billiet J y col. Analysis of phenotype and outcome in essential thrombocythemia with CALR or JAK2 mutations. Haematologica. 2015;100:893-897.
28. Levis M. FLT3 mutations in acute myeloid leukemia: what is the best approach in 2013? ASH Education Book. 2013; 2013:220-226.
29. Fritz A, Percy C, Jack A, Shanmugarathan S y col (Eds). International Classification of Diseases for Oncology (ICD-O) 3. Geneva World Health Organization. 2000.
30. Belli CB, Pinheiro RF, Bestach Y y col. Myelodysplastic syndromes in South America: a multinational study of 1080 patients. Am J Hematol. 2015;90:851-858.
31. Arbelbide J. Experiencia Argentina en síndromes mielodisplásicos. Hematología. 2016; 20:8-15. 32. Flores MG, Gonzalez JS, Nucifora E y col. Síndrome Mielodisplásico (SMD) con alteración del 5q. Característica y Evolución. Hematología. 2013, 17:145.
33. Basquiera A, Rivas M, Remaggi G y col. Allogeneic hematopoietic stem cell transplantation in adults with myelodysplastic syndrome: Experience of the Argentinean Group of Bone Marrow Transplantation (GATMO). Hematology. 2016; 21:162-169.
34. Smith BD. Myelodysplastic Syndromes: Challenges to Improving Patient and Caregiver Satisfaction. Am J Med. 2012; 125: S26–S30.
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