CAJ | 학술논문

目的评价骨髓增生异常综合征(MDS)WHO标准和MDS最低诊断标准对骨髓原始细胞比例不增高(<0.050)的MDS患者的诊断分型.方法回顾性收集1988年至2005年诊断的骨髓原始细胞比例不增高的210例MDS患者[FAB分型为难治性贫血(RA)或RA伴有环状铁粒幼红细胞(RARS)],重新分析细胞形态并采集相关实验室检查资料,用WHO标准和MDS最低诊断标准对这些患者进行诊断分型.结果按WHO分型标准进行诊断分型,其中RA 5例,RARS 7例,难治性血细胞减少伴有多系发育异常(RCMD)76例,RCMD和环状铁粒幼红细胞(RCMD-RS)9例,MDS不能分类(MDS-U)35例,5q-综合征3例,无法分型75例.WHO标准不能分型的患者中,16例骨髓二系或三系发育异常,而血细胞仅一系减少的患者随访半年后9例出现二系以上血细胞减少,可归为RCMD;对44例骨髓仅红系发育异常,血细胞二系减少或全血细胞减少无法进行分型患者,重新进行形态学分析,将那些尚未述及的细胞发育异常形态改变纳入判定该系细胞发育异常细胞计数范围后,27例患者粒系形态异常细胞≥0.100,可归入RCMD或RCMD-RS;15例三系均无细胞发育异常患者,采用MDS最低诊断标准诊断为MDS 5例,高度疑似MDS(HS-MDS)5例,意义未定的特发性血细胞减少(ICUS)5例.结论 MDS中只有很少一部分患者是真正意义上的RA;关于MDS的细胞发育异常形态学异常的范畴和量的界定值仍值得进一步探讨;MDS最低诊断标准是对WHO标准的很好的细化和补充.
목적 평개골수증생이상종합정(MDS)WHO표준화MDS최저진단표준대골수원시세포비례불증고(<0.050)적MDS환자적진단분형.방법 회고성수집1988년지2005년진단적골수원시세포비례불증고적210례MDS환자[FAB분형위난치성빈혈(RA)혹RA반유배상철립유홍세포(RARS)],중신분석세포형태병채집상관실험실검사자료,용WHO표준화MDS최저진단표준대저사환자진행진단분형.결과 안WHO분형표준진행진단분형,기중RA 5례,RARS 7례,난치성혈세포감소반유다계발육이상(RCMD)76례,RCMD화배상철립유홍세포(RCMD-RS)9례,MDS불능분류(MDS-U)35례,5q-종합정3례,무법분형75례.WHO표준불능분형적환자중,16례골수이계혹삼계발육이상,이혈세포부일계감소적환자수방반년후9례출현이계이상혈세포감소,가귀위RCMD;대44례골수부홍계발육이상,혈세포이계감소혹전혈세포감소무법진행분형환자,중신진행형태학분석,장나사상미술급적세포발육이상형태개변납입판정해계세포발육이상세포계수범위후,27례환자립계형태이상세포≥0.100,가귀입RCMD혹RCMD-RS;15례삼계균무세포발육이상환자,채용MDS최저진단표준진단위MDS 5례,고도의사MDS(HS-MDS)5례,의의미정적특발성혈세포감소(ICUS)5례.결론 MDS중지유흔소일부분환자시진정의의상적RA;관우MDS적세포발육이상형태학이상적범주화량적계정치잉치득진일보탐토;MDS최저진단표준시대WHO표준적흔호적세화화보충.
Objective To apply the WHO criteria and the minimal diagnostic criteria to the classification of myelodysplastic syndromes (MDS) with low percentage (< 0.050) bone marrow (BM) blasts. Methods Two hundred and ten MDS patients with less than 0.050 BM blasts diagnosed between 1988 and 2005 according to FAB criteria were retrospectively reclassified with WHO criteria(2001) and minimal diag-nostic criteria. Results According to the WHO criteria, 5 patients were diagnosed as refractory anemia (RA), 7 as refractory anemia with ringed sideroblasts(RARS), 76 as refractory cytopenia with muhilineage dysplasia(RCMD), 9 as RCMD-RS, 35 as MDS-unclassified(MDS-U), 3 as 5q - symdromes, and the rest 75 patients could not be classified suitably. Among the latter 75 patients 16 BM smears showed dysplasia in more than 2 cell lineage but only unilineage cytopenia in peripheral blood(PB). Nine of them were reclassified as RCMD after followed up for more than half a year. Forty-four BM smears showed erythroid dysplasia only, but bicytopenia or pancytopenia in PB. Twenty-seven of them were classified as RCMD after follow-up. Fifteen BM smears not showed dysplasia in any myeloid lineage were reclassified as MDS(5 patients), HS-MDS (5 patients) and idiopathic cytopenia of uncertain significance (ICUS) (5 patients) according to the MDS minimal diagnostic criteria. Conclusion According to WHO criteria(2001), RA is the least diagnosis in MDS. The minimal diagnostic criteria for MDS classification of patients not fulfilled the standard criteria of MDS.