Francesco Virdis, Sara Tacci, Federico Messina, Massimo Varcada, Department of Emergency General Surgery, Royal Free Hospital, London, Greater London NW3 2QG, United Kingdom.
World J Gastrointest Surg. 2013 Nov 27;5(11):306-8. doi: 10.4240/wjgs.v5.i11.306.
We present a case of a 19-year-old man with a 6-year history of Crohn's disease (CD), previously treated with 6-mercaptopurine, who was admitted to our department for Epstein-Barr virus (EBV) infection and subsequently developed a hemophagocytic lymphohistiocytosis (HLH). HLH is a rare disease which causes phagocytosis of all bone marrow derived cells. It can be a primary form as a autosomic recessive disease, or a secondary form associated with a variety of infections; EBV is the most common, the one with poorer prognosis. The incidence of lymphoproliferative disorders was increased in patients with inflammatory bowel disease (IBD) treated with thiopurines. Specific EBV-related clinical and virological management should be considered when treating a patient with IBD with immunosuppressive therapy. Moreover EBV infection in immunosuppressed patient can occur with more aggressive forms such as encephalitis and diffuse large B cell lymphoma. Our case confirms what is described in the literature; patients with IBD, particularly patients with CD receiving thiopurine therapy, who present 5 d of fever and cervical lymphadenopathy or previous evidence of lymphopenia should be screened for HLH.
我们报告了一例 19 岁男性患者，患有 6 年的克罗恩病（CD）病史，曾接受 6-巯基嘌呤治疗，因 EBV 感染入住我科，随后发展为噬血细胞性淋巴组织细胞增生症（HLH）。HLH 是一种罕见的疾病，会导致所有骨髓来源细胞的吞噬作用。它可以是一种作为常染色体隐性遗传疾病的原发性形式，也可以是一种与多种感染相关的继发性形式；EBV 是最常见的，预后较差。在接受硫嘌呤治疗的炎症性肠病（IBD）患者中，淋巴增生性疾病的发病率增加。在对接受免疫抑制治疗的 IBD 患者进行治疗时，应考虑特定的 EBV 相关临床和病毒学管理。此外，在免疫抑制患者中，EBV 感染可能以更具侵袭性的形式出现，如脑炎和弥漫性大 B 细胞淋巴瘤。我们的病例证实了文献中描述的内容；患有 IBD 的患者，特别是接受硫嘌呤治疗的 CD 患者，如果出现发热和颈部淋巴结病 5 天或以前有淋巴细胞减少的证据，应筛查 HLH。
