合并自身免疫异常的原发性骨髓增生异常综合征患者临床特征及生存分析

Clinical features and survival analysis in primary myelodysplastic syndromes patients with immuno- logical abnormalities

目的分析合并自身免疫异常的原发性骨髓增生异常综合征（MDS）患者的临床特征及生存。方法回顾性分析免疫相关检查完善或既往有自身免疫性疾病明确诊断病史的194例MDS患者临床资料并进行统计学分析。结果194例患者中37例（19．07％）合并自身免疫异常，其中16例（8．25％）例合并自身免疫性疾病，21例（10．82％）为无相关症状的免疫检查异常者。合并自身免疫性疾病组中〈60岁患者比例、女性比例、外周血CD4＋T细胞／CD8＋T细胞比值〈1者的比例、染色体异常为＋8者的比例显著高于免疫检查无异常组（P值均〈0．05）。合并自身免疫性疾病组患者2年预期生存率较免疫检查无异常组高，但差异无统计学意义（P=0．065）。无相关症状的免疫检查异常组中女性比例显著高于免疫检查无异常组（P〈0．05）。合并自身免疫性疾病组、无相关症状的免疫检查异常组与免疫检查无异常组相比，在年龄、诊断分型、IPSS预后分组、外周血血红蛋白、中性粒细胞计数、血小板计数、贫血严重程度、中性粒细胞减少程度、血清肿瘤坏死因子增高比例、染色体核型异常检出率、染色体核型预后分组、骨髓活检增生程度差异均无统计学意义（P值均〉0．05）。结论合并自身免疫性疾病的原发性MDS患者以女性、年龄〈60岁者为主，染色体异常为＋8的比例高，可能具有较好的预后。

Objective To analyze the clinical features and survival time in primary myelodysplatic syndromes（MDS） patients accompanied with immunological abnormalities. Methods The clinical informa- tion, laboratory findings and survival time in 194 untreated primary MDS patients with complete immunological laboratory tests or a past history of autoimmune disease were analyzed retrospectively. Results There were 37/194 cases（ 19.07% ）with autoimmune abnormalities, including 16/194 （8.25%） with autoimmune dis- ease and 21/194 asymptomatic cases（ 10.82% ） with serologic immunological abnormalities only. There was significant differences in the distribution of age 〈 60 years old, female, CD4 ~ T-cell/CD8 ~ T-cell ration 〈 1 and trisomy 8 （P 〈 0.05 ） between the cases with autoimmune disease and without autoimmune abnormalities. The former had a higher 2-year OS, but there was no significance （ P = O. 065 ）. There was no significant differences in the distribution of age, MDS-subtype, IPSS risk groups, haemoglobin, absolute neutrophil count, platelets count, the severity of anemia and neutropenia, high level of serologic TNF, chromosomal ab- normalities, cytogenetic risk groups and bone marrow cellularity（P 〉 0.05）. Conclusion MDS patients with autoimmune disease are mainly female and yonger than 60 years old , with high proportion of trisomy 8 and better prognosis.