Nayudu Suresh Kumar, Fida Nadia, Acidera Anna, Daniel Myrta, Rudikoff Donald, Niazi Masooma, Chilimuri Sridhar
Department of Medicine, Bronx Lebanon Hospital Center, Affiliated With Albert Einstein College of Medicine, Bronx, New York, USA.
Department of Pathology, Bronx Lebanon Hospital Center, Affiliated With Albert Einstein College of Medicine, Bronx, New York, USA.
World J Oncol. 2011 Jun;2(3):133-137. doi: 10.4021/wjon307w. Epub 2011 Jun 8.
Hemophagocytic lymphohistiocytosis (HLH) or Macrophage Activation Syndrome (MAS) is a potentially life threatening disorder that presents with fever, suppressed blood cell counts, hepatosplenomegaly and multi-organ failure. HLH has been reported in association with genetic mutations, infections, autoimmune disorders, and various malignancies. However to the best of our knowledge, HLH in association with infection has never been reported. We present a case of HLH in a patient with Epstein-Barr virus (EBV) positive natural killer T (NKT) cell cutaneous lymphoma and active infection. A 35-year-old male with recently diagnosed EBV positive NKT cell lymphoma was admitted for associated diarrhea. During the course of hospitalization he gradually developed pancytopenia and multi-organ failure leading to death. Post-mortem examination confirmed the diagnosis of hemophagocytic lymphohistiocytosis.
噬血细胞性淋巴组织细胞增生症（HLH）或巨噬细胞活化综合征（MAS）是一种潜在的危及生命的疾病，表现为发热、血细胞计数减少、肝脾肿大和多器官功能衰竭。HLH已被报道与基因突变、感染、自身免疫性疾病及各种恶性肿瘤有关。然而，据我们所知，从未有过与感染相关的HLH的报道。我们报告一例患有爱泼斯坦-巴尔病毒（EBV）阳性自然杀伤T（NKT）细胞皮肤淋巴瘤且伴有活动性感染的HLH患者。一名最近被诊断为EBV阳性NKT细胞淋巴瘤的35岁男性因相关腹泻入院。在住院期间，他逐渐发展为全血细胞减少和多器官功能衰竭，最终死亡。尸检确诊为噬血细胞性淋巴组织细胞增生症。
