You Ya-Hong, Meng Xian-Bin, Li Xing-Xin, Ge Mei-Li, Nie Neng, Huang Jin-Bo, Zhang Jing, Huang Zhen-Dong, Shao Ying-Qi, Shi Jun, Zheng Yi-Zhou
Institute of Hematology & Blood Disease Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Tianjin 300020, China.
Institute of Hematology & Blood Disease Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Tianjin 300020, China. E-mail:
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2017 Aug;25(4):1130-1135. doi: 10.7534/j.issn.1009-2137.2017.04.030.
To explore the clinical characteristic, therapeutic efficacy and prognosis of patients with hepatitis-associated aplasitc anemia (HAAA).
the clinical data and labrotatory examination results of 30 cases of HAAA were analyzed retrospectively, the 6-month response ratio and overall survival (OS) were assessed.
HAAA most commonly occured in males, with the occurence rate of males and females was 4:1, the median onset age was 16 (4-43) years old, HAAA oriented focus on sever aplastic anemia (SAA)(4 cases,13%) and very sever aplastic anemia (VSAA)(22 cases,73%). Aplastic anemia (AA) could be seen on occurence of hepatitis (accompanied aplastic anemia) (7 cases,23%), or after the onset of hepatits (delayed aplastic anemia) (23 cases,77%), but more often occured in the latter. Statistical analysis showed that when compared with the patients of delayed aplastic anemia, patients accompanied aplastic anemia possesses lower levels of glutamic-pyruvic transaminase(ALT), aspertate aminotransferase (AST) and total bilirubin (TBIL)(P=0.042,0.012,0.001), and possessed a more obvious lymphoid cell disorder when AA occured, with more lower peripheral blood CD19 B cells proportion (P=0.046) and more obvious imbalance of CD4/CD8 ratio, but the difference was no statistical significant (P=0538). Factors affecting the 6-month respose were the severity of AA (P=0.044), the peak level of bilirubin of hepatitis (P=0.006) and the propotion of mature monocyte in bone marrow (P=0.034). The long-term follow-up showed that the 2-year OS of HAAA was 64.3±9.2%, the 6-month curative efficacy significantly affect the prognosis (P<0.001).
HAAA more often occur in young male, HAAA is mainly SAA and VSAA and mostly non-A-C hepatitis associated aplastic anemia, patients usually have a high incidence of early infection. Patients acompanied with aplastic anemia possess more obvious immunological derangement; the treatment efficacy for HAAA is poor, patients who haven't obtained 6-month response indicate a sinister prognosis, allogeneic hematopoietic stem cell transplantion is a better choice for these patients.
探讨肝炎相关性再生障碍性贫血（HAAA）患者的临床特征、治疗效果及预后。
回顾性分析30例HAAA患者的临床资料及实验室检查结果，评估6个月缓解率及总生存期（OS）。
HAAA最常见于男性，男女发病率为4:1，中位发病年龄为16（4 - 43）岁，HAAA主要集中于重型再生障碍性贫血（SAA）（4例，13%）和极重型再生障碍性贫血（VSAA）（22例，73%）。再生障碍性贫血（AA）可在肝炎发生时出现（伴发再生障碍性贫血）（7例，23%），或在肝炎发病后出现（迟发性再生障碍性贫血）（23例，77%），但以后者更为常见。统计分析显示，与迟发性再生障碍性贫血患者相比，伴发再生障碍性贫血患者的谷丙转氨酶（ALT）、谷草转氨酶（AST）和总胆红素（TBIL）水平较低（P = 0.042、0.012、0.001），且AA发生时淋巴细胞紊乱更明显，外周血CD19 B细胞比例更低（P = 0.046），CD4/CD8比值失衡更明显，但差异无统计学意义（P = 0.538）。影响6个月缓解的因素为AA的严重程度（P = 0.044）、肝炎胆红素峰值水平（P = 0.006）及骨髓中成熟单核细胞比例（P = 0.034）。长期随访显示，HAAA的2年OS为64.3±9.2%，6个月疗效显著影响预后（P < 0.001）。
HAAA多见于年轻男性，主要为SAA和VSAA，大多为非A - C型肝炎相关性再生障碍性贫血，患者早期感染发生率高。伴发再生障碍性贫血患者免疫紊乱更明显；HAAA治疗效果差，未获得6个月缓解的患者预后凶险，异基因造血干细胞移植是这些患者较好的选择。
