Abstract

7岁4月龄患儿因“间断发热1年7月余,活动后气促、发绀2个月”入院,临床表现为肺动脉高压、淋巴结及肝脾肿大。EB抗体异常、血浆及全血EB病毒DNA升高、颈部淋巴结活检示EB病毒感染相关淋巴增殖性疾病,诊断为慢性活动性EB病毒感染合并肺动脉高压(PAH)。予左旋培门冬酰胺酶+脂质体阿霉素+依托泊苷+甲泼尼龙(L-DEP)方案化疗后肺动脉压力降低并进行异基因造血干细胞移植,移植后64 d PAH消失,随访至移植后10个月,病情未复发。.

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