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KMID : 0366220070420020071
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Korean Journal of Hematology
2007 Volume.42 No. 2 p.71 ~ p.90
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The Clinical Guidelines for Myelodysplastic Syndrome
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Cheong June-Won
Kook Hoon
Bang Su-Mi
Lee Je-Hwan
Joo Young-Don
Kim In-Ho
Kim Hyeoung-Joon
Park Chan-Jeoung
Park Hyeon-Jin
Ahn Jin-Seok
Yoon Sung-Soo
Won Jong-Ho
Lee Hong-Ki
Jung Chul-Won
Jo Deog-Yeon
Cho Bin
Han Kyoung-Ja
Min Yoo-Hong
Kim Sun-Hee
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Abstract
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The myelodysplastic syndromes (MDS) are characterized by ineffective hematopoiesis associated with multilineage cytopenias leading to serious morbidity or mortality, and the additional risk of leukemic transformation. The management of patients with MDS can be very complex and varies according to both the clinical manifestations in individual patients as well as the presence of complicating medical conditions. However, therapeutic dilemmas still exist for MDS due to the multifactorial pathogenetic features of the disease, its heterogeneous stages, and the elderly patient population. For these reasons, proper guidelines for management are necessary. This review describes the proper diagnosis for MDS, decision-making approaches for optimal therapeutic options that are based on a consideration of patient clinical factors and risk-based prognostic categories, and the use of recently available biospecific drugs such as hypomethylating agents that are potentially capable of abrogating the abnormalities associated with MDS. Proper indications and methods for transplantation, response criteria, management for iron overload for highly transfused patients and specific considerations for MDS in childhood are also described. All of these topics were discussed at the third symposium of AML/MDS working party on 3 March, 2007. (Korean J Hematol 2007;42:71-90.)
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KEYWORD
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Myelodysplastic syndrome, Guideline, Diagnosis, Hypomethylating agents, Transplantation, Response criteria, Iron chelating treatment, MDS in childhood
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