10例意义未明特发性血细胞减少症的临床特征研究  

Stady on the clinical characteristics of 10 patients with idiopathic cytopenia of undetermined significance

作　　者：浦杰[1] 石军[2] 陈真 陆道培 浦权[2,3] 

机构地区：[1]桂林医学院附属医院血液科,广西桂林541001 [2]上海市第六人民医院血液科,200233 [3]上海市市东医院病理科,200438 [4]上海市道培医院,200240

出　　处：《重庆医学》2014年第21期2698-2700,共3页Chongqing medicine

基　　金：上海科学技术委员会平台项目(13DZ2293702)

摘　　要：目的：探讨意义未明特发性血细胞减少症（ICUS）患者的临床和病理学特征，并提供诊断依据。方法选择未达骨髓增生异常综合征（MDS）最低诊断标准，但存在持久（＞6个月）血细胞减少，骨髓三系细胞无明显病态发育和异常核型证据而考虑诊断为ICUS的患者10例，对其临床和病理学特征进行分析。结果10例患者中的2例患者转型至MDS，骨髓复查显示大于或等于二系细胞出现诊断性病态发育，染色体核型分析1例仍正常，另1例显示丢失1个正常20号染色体（20q-）。8例随访6～25个月，至今仍维持ICUS原状。结论未达MDS最低诊断标准的ICUS患者，必须进行定期随访；部分患者经一定潜伏期后可转型至公开的MDS；之前实属Pre-MDS（MDS-早期），表明ICUS应该引起临床高度重视，避免漏诊。Objective To analyze the clinical and pathologic features in patients with ldiopathic cytopenia of undetermined signif-icance （ICUS） ,and provide the diagnostic basis .Methods 10 ICUS patients who did not fulfil minimal diagnostic criteria for MDS but suffered from constant （〉6 months） progressive cytopenia ,with no marked dysplasia （〈10% in three-major cell lineages） and abnormal karyotype were reported .Results 2 of 10 patients transited to MDS ,bone marrow review showed that ≥2 cells appeared diagnostic pathology development .Karyotype analysis showed that 1 case was still normal ,1 case lost a normal chromosome 20 （20q-） ,the rest 8 cases had been efollowed up for 6 to 25 months and still maintain ICUS .Conclusion Patients with MDS who fail to meet the diagnostic criteria of ICUS ,must carry out regular follow-up .There are some patients can transition to public MDS after a certain incubation period ,in fact it is Pre-MDS or MDS-early before ,which shows that ICUS should cause clinical attention ,so that avoid missed diagnosis .

关 键 词：骨髓增生异常综合征 意义未明特发性血细胞减少症 最低标准 核型 

分 类 号：R552[医药卫生—血液循环系统疾病]