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目的报告表现酷似重型再生障碍性贫血(sAA)的急性造血停滞(AAH)患者临床特征,探讨与 SAA 和极重型再生障碍性贫血(vSAA)患者的鉴别。方法以1111例初治 SAA 和 vSAA 患者为对照,回顾性分析23例临床表现酷似再生障碍性贫血的 AAH 患者临床及实验室特征。结果 23例 AAH 中16例临床及实验室检查符合 SAA 或 vSAA 诊断标准,自发病起2个月内出现自发性造血恢复,中位时间为17(8～40)d。与对照组患者比较,符合 SAA 或 vSAA 诊断标准的16例 AAH 患者年龄大[中位年龄35.5岁,SAA(包括 vSAA)为21.0岁],多伴有其他疾病及用药史,常以发热症状起病;实验室检查与 SAA 或 vSAA 相似,但低血清白蛋白发生率高,总铁结合力减低更为常见,骨髓 CFU-GM 相对较高。结论某些 AAH 与 SAA 或 vSAA 临床表现非常相似,常需回顾性诊断方可鉴别,分析其起病情况及实验室特征有助于早期识别。
Objective To report the clinical features of acute hematopoietic arrest (AAH) patients who resemble severe acute aplastic anemia (sAA) and to identify patients with SAA and very severe aplastic anemia (vSAA). Methods 1111 patients with newly diagnosed SAA and vSAA were retrospectively analyzed. The clinical and laboratory features of 23 AAH patients with clinical manifestations resembling aplastic anemia were retrospectively analyzed. Results The clinical and laboratory findings of 16 cases of AAH met the diagnostic criteria of SAA or vSAA. Spontaneous hematopoiesis recovered within 2 months after onset, with a median time of 17 (8 ~ 40) days. Compared with patients in the control group, 16 patients with AAH who met the diagnostic criteria of SAA or vSAA were older (median age 35.5 years, SAA (including vSAA) 21.0 years), more often accompanied by other diseases and medication history, often with fever symptoms The laboratory tests were similar to those of SAA or vSAA, but the high incidence of low serum albumin and total iron-binding decreased more commonly with higher bone marrow CFU-GM. Conclusion Some of the AAH and SAA or vSAA clinical manifestations are very similar, often requiring a retrospective diagnosis before identification, analysis of its onset and laboratory characteristics contribute to early identification.