Booth A L, Osehobo P, Rodgers-Soriano D, Lalarukh A, Eltorky M A, Stevenson H L
University of Texas Medical Branch, Galveston, Texas, USA.
WellStar Spalding/Sylvan Grove Hospital, Griffin, Georgia, USA.
Case Rep Gastroenterol. 2018 Apr 24;12(1):99-108. doi: 10.1159/000486190. eCollection 2018 Jan-Apr.
Hemophagocytic lymphohistiocytosis (HLH) is an uncommon disease that often presents with nonspecific findings. A high index of suspicion is necessary to make a prompt diagnosis and prevent fatal disease. A 45-year-old man presented with fever, hypotension, abdominal pain, nausea, and vomiting. Imaging showed hepatosplenomegaly and laboratory tests revealed pancytopenia, increased ferritin, and a cholestatic pattern of injury with elevated alkaline phosphatase and total bilirubin. Due to a history of Crohn disease, systemic lupus erythematous, and rheumatoid arthritis, the patient was on immunosuppressants, including infliximab. After multiple negative cultures, persistent fever, and days of empiric broad spectrum antibiotics, our differential shifted to fever of unknown origin. A liver wedge biopsy revealed areas of sinusoidal dilatation with enlarged, activated macrophages containing erythrocytes and intracytoplasmic iron, consistent with hemophagocytosis due to HLH. The portal tracts showed mixed lymphoplasmacytic inflammation, a prominent bile ductular reaction, periportal fibrosis, and scattered large cells with occasional binucleation and prominent nucleoli. These cells stained positive for Epstein-Barr virus encoding region in situ hybridization, PAX5, CD15, and CD30, and hepatic involvement by classic Hodgkin lymphoma was diagnosed and determined to be the cause of the HLH and cholestatic pattern of injury. Simultaneously, a bone marrow biopsy showed diffuse involvement by Hodgkin lymphoma with a similar staining pattern. Aggressive treatment failed and the patient succumbed to multiorgan failure. HLH is a rare, potentially fatal disease, with nonspecific signs and symptoms, and should be considered in any patient presenting with fever and pancytopenia, especially if they are immune compromised.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见疾病，常表现为非特异性症状。高度怀疑对于及时诊断和预防致命疾病至关重要。一名45岁男性出现发热、低血压、腹痛、恶心和呕吐。影像学检查显示肝脾肿大，实验室检查显示全血细胞减少、铁蛋白升高以及碱性磷酸酶和总胆红素升高的胆汁淤积性损伤模式。由于有克罗恩病、系统性红斑狼疮和类风湿关节炎病史，该患者正在使用包括英夫利昔单抗在内的免疫抑制剂。在多次培养结果为阴性、持续发热且经验性使用广谱抗生素数天后，我们的鉴别诊断转向不明原因发热。肝脏楔形活检显示窦状隙扩张区域，有含有红细胞和胞质内铁的增大、活化巨噬细胞，符合HLH所致噬血细胞现象。汇管区可见混合性淋巴浆细胞炎症、明显的胆小管反应、汇管区周围纤维化以及散在的大细胞，偶见双核和明显核仁。这些细胞在爱泼斯坦-巴尔病毒编码区原位杂交、PAX5、CD15和CD30染色中呈阳性，诊断为经典型霍奇金淋巴瘤累及肝脏，并确定其为HLH和胆汁淤积性损伤模式的病因。同时，骨髓活检显示霍奇金淋巴瘤弥漫性累及，染色模式相似。积极治疗失败，患者死于多器官功能衰竭。HLH是一种罕见的、潜在致命的疾病，症状和体征不具特异性，对于任何出现发热和全血细胞减少的患者都应考虑到该病，尤其是免疫功能低下者。
