Yang W R, Jing L P, Zhou K, Peng G X, Li Y, Ye L, Li Y, Li J P, Fan H H, Song L, Zhao X, Yang Y, Zhang F K, Zhang L
Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.
Zhonghua Xue Ye Xue Za Zhi. 2016 May 14;37(5):399-404. doi: 10.3760/cma.j.issn.0253-2727.2016.05.009.
To analyze the clinical characteristics and to evaluate immunosuppressive therapy (IST) response and survival in hepatitis-associated aplastic anemia (HAAA).
We retrospectively analyzed clinical characteristics, IST response, long-term survival and clonal evolution in 41 HAAA patients, and compared those with age and bone marrow failure matched idiopathic aplastic anemia (IAA) patients.
The prevalence of HAAA among cases of SAA was 4.34% (41/944). The proportion of VSAA in HAAA cases was significantly higher than IAA (65.9% vs 39.4%, P=0.001). There was no significant difference in the prevalence of hemorrhage and infections between HAAA and IAA patients, but the duration of infection persistence in HAAA group was much longer than IAA group [21 (4-100) d vs 13 (3-139) d, P=0.048]. The absolute counts of CD3(+) T-cell, CD3(+)CD4(+)T-cell, CD3(+)CD8(+)T-cell and ratio of CD4(+) T-cell/CD8(+) T-cell in HAAA were significant lower than that in IAA patients. However, the percentage of CD3(+)CD8(+)T-cell in HAAA was significant higher than that in IAA (P <0.05). The total response in HAAA and IAA patients treated with IST were 34.1% vs 34.1% (P=1.000), 56.1% vs 53.7% (P=0.787), and 73.2% vs 68.3% (P=0.558) at 3, 6, 12 months after IST, respectively. There were no significant difference in 5-year overall survival and event-free survival between HAAA and IAA patients (90% vs 87.1%, P=0.700; 71.9% vs 62.4%, P=0.450).
HAAA was a rare distinct variant of aplastic anemia with more severe bone marrow failure and more severe imbalance of the T cell immune system than IAA. Treatment outcomes were comparable in patients with HAAA and IAA.
分析肝炎相关性再生障碍性贫血(HAAA)的临床特征,评估免疫抑制治疗(IST)反应及生存情况。
我们回顾性分析了41例HAAA患者的临床特征、IST反应、长期生存及克隆演变情况,并与年龄及骨髓衰竭相匹配的特发性再生障碍性贫血(IAA)患者进行比较。
重型再生障碍性贫血(SAA)病例中HAAA的患病率为4.34%(41/944)。HAAA病例中极重型再生障碍性贫血(VSAA)的比例显著高于IAA(65.9%对39.4%,P = 0.001)。HAAA和IAA患者出血和感染的患病率无显著差异,但HAAA组感染持续时间远长于IAA组[21(4 - 100)天对13(3 - 139)天,P = 0.048]。HAAA患者CD3(+) T细胞、CD3(+)CD4(+)T细胞、CD3(+)CD8(+)T细胞的绝对计数及CD4(+) T细胞/CD8(+) T细胞比值均显著低于IAA患者。然而,HAAA患者CD3(+)CD8(+)T细胞百分比显著高于IAA患者(P <0.05)。IST治疗后3、6、12个月时,HAAA和IAA患者的总反应率分别为34.1%对34.1%(P = 1.000)、56.1%对53.7%(P = 0.787)、73.2%对68.3%(P = 0.558)。HAAA和IAA患者5年总生存率和无事件生存率无显著差异(90%对87.1%,P = 0.700;71.9%对62.4%,P = 0.450)。
HAAA是再生障碍性贫血中一种罕见的独特类型,与IAA相比,骨髓衰竭更严重,T细胞免疫系统失衡更严重。HAAA和IAA患者的治疗结果相当。