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 Iymphoproliferative disease after hematopoietic stem cell transplantation
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.
更多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 multifocal 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.
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