造血干细胞移植后淋巴细胞增殖性疾病伴EB病毒相关肺炎  

Epstein-Barr virus-associated pneumonia in patients with post-transplant lymphoproliferative disease after hematopoietic stem cell transplantation

作　　者：刘启发[1] 范志平[1] 罗晓丹[1] 张钰[1] 孙竞[1] 

机构地区：[1]南方医科大学南方医院血液科,广州510515

出　　处：《中华医学杂志》2008年第44期3123-3126,共4页National Medical Journal of China

基　　金：国家高技术研究发展计划基金资助项目（2006AA02Z4A0）;广东省自然科学基金资助项目（7005160）

摘　　要：目的调查造血干细胞移植（HSCT）后EB病毒（EBV）再活化或感染诱导（PTLD）和EBV相关肺炎。方法统计2001年1月至2007年12月323例接受HSCT后患者PTLD和PTLD伴EBV相关肺炎发生，PTLD伴EBV相关肺炎的临床表现。结果323例HSCT后患者7例发生PTLD，包括弥漫性大B细胞淋巴瘤5例、多形性B细胞增生1例和外周非特殊T细胞淋巴瘤1例；7例患者均以腔外淋巴结肿大为首发症状，3例伴有EBV相关肺炎。3例EBV相关肺炎患者均有发热和呼吸困难，疾病进展迅速，均在PTLD发病后2周内因呼吸衰竭而死亡，其中包括1例多脏器功能衰竭。肺部CT表现：多灶性斑状和弥漫性毛玻璃状影；支气管肺泡灌洗液（BAL）EBV—DNA阳性，细胞学分析主要为CD3＋T细胞、CD19＋和CD20＋B细胞缺乏；肺组织活检：间质和肺泡主要为CD3＋T细胞浸润、部分为CD68＋巨噬细胞。结论造血干细胞移植后EBV相关PTLD患者伴EBV相关肺炎并不罕见，疾病进展迅速；BAL细胞学分析和肺组织活检有助于诊断。Objective To investigate the clinical features of Epstein-Barr virus （EBV） reactivation or infection-induced post-transplant lymphoproliferative disease （PTLD） and EBV-associated pneumonia after hematopoietic stem cell transplantation （HSCT）. Methods The clinical data of 7 patients with PTLD, 6 from the 239 patients undergoing allo-HSCT and 1 from the 84 patients undergoing auto-HSCT, were analyzed. Results All the 7 patients had extravisceral lymph node enlargement as the primary presentation. Five cases were diagnosed as with diffuse large B-cell lymphoma, 1 case as with polymorphic B-cell hyperplasia, and 1 case peripheral T-cell lymphoma-unspecified. Sex chromosome analysis showed that the tumor cells originated from the donors. ELISA showed that plasma EBV DNA was positive in 6 of the 7 patients and was negative in 1 patient. Chest CT revealed muhifocal patches and diffuse ground-glass attenuation in both lungs. EBV-DNA was positive in the bronchoalveolar lavage （BAL） fluid. The T cells in all of the BAL fluid were mainly CD3 ＋ T cells without CD19 ＋ and CD20 ＋ B cells. Lung biopsy showed interstitial and intra-alveolar infiltration, constituted mainly by CD3 ＋ T cells and partly by CD68 ＋ macrophages, however, without CD19 ＋ and CD20 ＋ B cells. The 3 patients with PTLD accompanied by EBV associated-pneumonia had hyperpyrexia and dyspnea, and their condition aggravated rapidly and eventually all the 3 patients died of respiratory failure, of which 1 case with multiple organ failure died within 2 weeks since the onset of PTLD. Conclusions EBV-associated PTLD accompanied by EBV-associated pneumonia is not rare and is always severe. Cytology of BAL fluid and lung biopsy help diagnose.

关 键 词：淋巴组织增殖性疾病 造血干细胞移植 EB病毒 肺炎 

分 类 号：R686[医药卫生—骨科学]