淋巴瘤相关噬血细胞综合征的临床诊治和预后:3例报道及文献复习

The clinical diagnosis, treatment and prognosis of lymphoma-associated hemophagocytic syndrome: A report of three cases and literature review

目的 :通过分析淋巴瘤相关噬血细胞综合征(lymphoma-associated hemophagocytic syndrome,LAHS)患者的病史资料,提高对LAHS临床诊治和预后因素的认识。方法 :回顾性分析2013年4月—8月南昌大学第一附属医院血液科收治的3例LAHS患者的临床病历资料,并结合相关文献进行复习。结果 :3例患者均符合噬血细胞综合征(hemophagocytic syndrome,HPS)诊断标准,其中2例原发病为外周T细胞淋巴瘤,1例为侵袭性自然杀伤细胞白血病。3例患者均在骨髓涂片或活检组织中观察到噬血现象,1例患者伴有EB病毒感染。2例患者生存期仅27 d和31 d,另1例患者行大剂量甲基强的松龙+E-CHOP方案治疗后至今存活65 d。结论 :LAHS起病凶险,病死率极高,临床上容易和病因不明或EB病毒相关的HPS混淆,应积极甚至反复取材获取淋巴瘤证据。治疗上应同时考虑控制高炎性反应因子风暴和针对淋巴瘤本病治疗。

Objective： To improve understanding of clinical diagnosis, treatment and prognosis of lymphoma-associated hemophagocytic syndrome （LAHS） through retrospective review of medical records of three patients with LAHS and literature review. Methods： Three patients with LAHS hosptalized in Department of Hematology, First Affiliated Hospital of Nanchang University between April and August 2013 were collected and their medical records were retrospectively analyzed. A review of related literatures was also performed. Results： All three cases met the diagnostic criteria of hemophagocytic syndrome （HPS）. Of the three patients, two patients had a primary disease of peripheral T-cell lymphoma and one had a primary disease of aggressive natural killer cell leukemia. Haemophagocytic phenomenon was evidenced by bone marrow aspiration or bone marrow biopsy in these three patients. One patient was infected with Epstein-Barr virus （EBV）. One patient treated by high-dose methylprednisolone in combination with E-CHOP regimen has survived for 65 days so far, whereas the other two patients only survived for 27 and 31 days, respectively. Conclusion： LAHS is a disease with a rapid-onset and a high mortality, and can be easily confused with agnogenic or EBV-associated HPS. The evidence of lymphoma should be obtained through repeated biopsies. Controling the cytokine strom and chemotherapy for lymphoma may be more suitable for patients with LAHS.