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免疫抑制疗法治疗血清阴性肝炎相关性再生障碍性贫血的疗效与安全性。

Efficacy and safety of immunosuppressive therapy in the treatment of seronegative hepatitis associated aplastic anemia.

作者信息

Chen Hai-Fei, Xu Bin-Xian, Shen Hong-Shi, Li Zheng-Yang, Jin Ling-Juan, Tang Jie-Qing, Wang Jing, Zhu Jing-Jing, Qin Long-Mei, Cui Qing-Ya, Ren Yong-Ya, Wu Tian-Qin

机构信息

Department of Hematology, 100th Hospital of People's Liberation Army, Suzhou, Jiangsu province, The People's Republic of China.

出版信息

Drug Des Devel Ther. 2014 Sep 9;8:1299-305. doi: 10.2147/DDDT.S67908. eCollection 2014.

Abstract

OBJECTIVE

To investigate the clinical characteristics of seronegative hepatitis-associated aplastic anemia (AA) (SNHAA) and hepatitis B virus (HBV) infection complicating AA (HBVAA), and thereby compare the efficacy of immunosuppressive therapy (IST).

METHODS

An analysis was conducted on the clinical data of ten patients with SNHAA out of 332 cases of AA from our center at AA diagnosis, and on the efficacy of IST. This was compared to 22 cases of HBVAA at AA onset as well as the associated IST outcomes.

RESULTS

Nine patients with SNHAA developed severe aplastic anemia, with a median age of 18 years. After IST, six (60%) of the SNHAA patients achieved complete remission and two achieved partial remission. The patients with HBVAA had a total response rate of 82.3%. The disease recurred in two HBVAA patients. No statistically significant differences were observed in response rate, mortality, and recurrence rate between both groups. As compared with HBVAA, patients with SNHAA had a shorter interval from the acute episode of hepatitis to AA onset (4 months versus 92 months, P=0.00), a quicker response to IST (2.5 months versus 4.5 months, P=0.018), a lower proportion of bone marrow hematopoietic tissues (20.6% versus 23.6%, P=0.03), and lower white blood cell and absolute neutrophil count (0.8 × 10(9)/L versus 1.23 × 10(9)/L and 0.26 × 10(9)/L versus 0.58 × 10(9)/L, P=0.026 and P=0.0009, respectively). No significant liver damage or hepatitis B fulminant infection was observed in either group during the follow-up.

CONCLUSION

The prevalence of SNHAA is 3.01%. SNHAA often presents as severe AA and responds to IST quickly. Neither hepatitis prior to AA nor AA complicating HBV infection have been shown to influence the early efficacy of IST and adverse events, and HBV may not be the causative agent of AA.

摘要

目的

探讨血清阴性肝炎相关性再生障碍性贫血(SNHAA)及乙型肝炎病毒(HBV)感染合并再生障碍性贫血(HBVAA)的临床特征,进而比较免疫抑制治疗(IST)的疗效。

方法

对本中心332例再生障碍性贫血患者中确诊为SNHAA的10例患者的临床资料及IST疗效进行分析,并与再生障碍性贫血发病时为HBVAA的22例患者及其相关IST结果进行比较。

结果

9例SNHAA患者发生重型再生障碍性贫血,中位年龄为18岁。IST治疗后,6例(60%)SNHAA患者达到完全缓解,2例达到部分缓解。HBVAA患者的总缓解率为82.3%。2例HBVAA患者疾病复发。两组在缓解率、死亡率和复发率方面未观察到统计学显著差异。与HBVAA相比,SNHAA患者从肝炎急性发作到再生障碍性贫血发病的间隔时间更短(4个月对92个月,P = 0.00),对IST的反应更快(2.5个月对4.5个月,P = 0.018),骨髓造血组织比例更低(20.6%对23.6%,P = 0.03),白细胞和绝对中性粒细胞计数更低(0.8×10⁹/L对1.23×10⁹/L以及0.26×10⁹/L对0.58×10⁹/L,P分别为0.026和0.0009)。随访期间两组均未观察到明显肝损伤或乙型肝炎暴发性感染。

结论

SNHAA的患病率为3.01%。SNHAA常表现为重型再生障碍性贫血且对IST反应迅速。再生障碍性贫血之前的肝炎以及再生障碍性贫血合并HBV感染均未显示会影响IST的早期疗效及不良事件,且HBV可能不是再生障碍性贫血的致病因素。

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