Hofstrand Reese, Casey Bradley, Onyeaso Elizabeth
Internal Medicine, Cape Fear Valley Medical Center, Fayetteville, USA.
Internal Medicine/Infectious Disease, Cape Fear Valley Medical Center, Fayetteville, USA.
Cureus. 2023 Jul 12;15(7):e41779. doi: 10.7759/cureus.41779. eCollection 2023 Jul.
Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome in which widespread activation of the immune system causes multi-organ tissue damage. HLH is a serious and potentially fatal disorder characterized by excessive immune system activation. It is characterized by a dysregulation in natural killer (NK) T-cell function, causing activation of lymphocytes and histiocytes. These cells secrete a large number of inflammatory cytokines and infiltrate various tissues causing multi-organ system failure. The spectrum consists of hereditary or "primary" HLH that comprises genetically heterogeneous conditions, occurring during childhood. The secondary form presents later in life and is associated with several conditions mainly malignancy, autoimmune diseases, viral or bacterial infections, and hematological diseases. Here we present an interesting case in which a 39-year-old patient presented with a complaint of shortness of breath. He was diagnosed with obstructive uropathy in the emergency department and subsequently developed acute liver injury, acute kidney injury, bacteremia, and was diagnosed with HLH with comorbid bacteremia.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的综合征，免疫系统的广泛激活会导致多器官组织损伤。HLH是一种严重且可能致命的疾病，其特征是免疫系统过度激活。它的特点是自然杀伤（NK）T细胞功能失调，导致淋巴细胞和组织细胞激活。这些细胞分泌大量炎性细胞因子并浸润各种组织，导致多器官系统衰竭。该谱系包括遗传性或“原发性”HLH，其包括基因异质性疾病，发生于儿童期。继发性形式在生命后期出现，与多种疾病相关，主要是恶性肿瘤、自身免疫性疾病、病毒或细菌感染以及血液系统疾病。在此，我们呈现一个有趣的病例，一名39岁患者主诉呼吸急促。他在急诊科被诊断为梗阻性尿路病，随后发展为急性肝损伤、急性肾损伤、菌血症，并被诊断为合并菌血症的HLH。
