CAJ | 학술논문

骨髓增生异常综合征(myelodysplastic syndromes, MDS)是起源于造血干细胞的一组异质性髓系克隆性疾病，特点是髓系细胞分化及发育异常，表现为无效造血、难治性血细胞减少、造血功能衰竭，高风险向急性髓系白血病（AML）转化。MDS治疗主要解决两大问题：骨髓衰竭及并发症、AML转化。就患者群体而言，M D S患者自然病程和预后的差异性很大，治疗宜个体化。它不等同于“白血病前期”，是从回顾性研究白血病前期中界定出来的一个独立综合征。主要临床特征是骨髓病态无效造血、外周血细胞减少、治疗反应差和高风险发展为急性白血病。约20％～40％最终转化为急性白血病，M D S的发生率为5/100000，发病以老年人为主，少数为儿童。近年来，MDS特别是低增生型骨髓增生异常综合征(HMDS)发病率较以前有所提高，且不易诊断和鉴别诊断。故在此对其病因、分型及实验室检查做一简单综述，望对临床诊断和鉴别诊断有所帮助。
골수증생이상종합정(myelodysplastic syndromes, MDS)시기원우조혈간세포적일조이질성수계극륭성질병，특점시수계세포분화급발육이상，표현위무효조혈、난치성혈세포감소、조혈공능쇠갈，고풍험향급성수계백혈병（AML）전화。MDS치료주요해결량대문제：골수쇠갈급병발증、AML전화。취환자군체이언，M D S환자자연병정화예후적차이성흔대，치료의개체화。타불등동우“백혈병전기”，시종회고성연구백혈병전기중계정출래적일개독립종합정。주요림상특정시골수병태무효조혈、외주혈세포감소、치료반응차화고풍험발전위급성백혈병。약20％～40％최종전화위급성백혈병，M D S적발생솔위5/100000，발병이노년인위주，소수위인동。근년래，MDS특별시저증생형골수증생이상종합정(HMDS)발병솔교이전유소제고，차불역진단화감별진단。고재차대기병인、분형급실험실검사주일간단종술，망대림상진단화감별진단유소방조。
Myelodysplastic syndrome ( myelodysplastic syndromes, MDS ) is at the origin of hematopoietic stem cel s by a group of heterogeneous myeloid clonal disorders, characterized by myeloid cel differentiation and developmental abnormalities, manifested as ineffective hematopoiesis, refractory cytopenia, hematopoietic function failure, to high risk acute myeloid leukemia ( AML ) transformation. MDS treatment mainly solve two big problems: bone marrow failure and complications, the conversion of AML. The patient group, natural course and prognosis of patients with MDS differences greatly, the treatment should be individualized. It does not equate to " preleukemia ", is from a retrospective study of preleukemia defined from an independent syndrome. The main clinical features are bone marrow pathological ineffective hematopoiesis, peripheral blood cel reduction, differential treatment response and high risk for developing acute leukemia. About 20%~40% final transformation into acute leukemia, the incidence rate of MDS was 5/100 000, the incidence in the elderly, a few children. In recent years, especial y MDS hypoplastic myelodysplastic syndrome ( HMDS ) incidence than before the increase, and not easy to diagnosis and differential diagnosis. This is the etiology, classification and laboratory examination to do a simple overview, we hope to help clinical diagnosis and differential diagnosis.