From the Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota.
J Neuropathol Exp Neurol. 2020 Aug 1;79(8):915-920. doi: 10.1093/jnen/nlaa061.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal disease if not diagnosed and treated promptly. HLH can be due to genetic factors or infections, malignancies and collagen-associated vascular diseases. Malignancy-associated HLH is not only more common in the setting of T/NK-cell lymphomas, but may also rarely be seen in the setting of B-cell lymphoma. Here, we describe a unique case of a patient who initially was diagnosed with HLH secondary to Epstein Barr virus (EBV) infection and subsequently developed EBV-positive diffuse large B-cell lymphoma affecting the brain. This case highlights the spectrum of findings associated with EBV infections and the challenges in diagnosing underlying diseases associated with HLH.
噬血细胞性淋巴组织细胞增生症(HLH)如果不能及时诊断和治疗,是一种罕见且常常致命的疾病。HLH 可由遗传因素或感染、恶性肿瘤和胶原血管疾病引起。与恶性肿瘤相关的 HLH不仅在 T/NK 细胞淋巴瘤中更为常见,而且在 B 细胞淋巴瘤中也可能罕见地出现。在此,我们描述了一例独特的患者,该患者最初被诊断为 EBV 感染继发 HLH,随后发展为累及大脑的 EBV 阳性弥漫性大 B 细胞淋巴瘤。该病例突出了 EBV 感染相关的一系列表现,并强调了诊断 HLH 相关潜在疾病的挑战。