Melki Gabriel, Alkomos Mina Fransawy, Nanavati Sushant, Kumar Vinod, Mariyam Christina, Maroules Michael
St. Joseph's University Medical Center, Internal Medicine Department, Paterson, NJ, USA.
St. Joseph's University Medical Center, Pulmonary and Critical Care Department, Paterson, NJ, USA.
Autops Case Rep. 2021 May 6;11:e2021285. doi: 10.4322/acr.2021.285. eCollection 2021.
Acquired Hemophagocytic Lymphohistiocytosis is a rare and deadly syndrome resulting from an overactive immune system, with uncontrolled activation of macrophages and lymphocytes, hypercytokinemia, and systemic inflammatory response. A 75-year-old male presented with typical anginal pain and was diagnosed with the acute coronary syndrome, which required a percutaneous transluminal coronary angioplasty. Instead of resolving the symptoms, the patient began to exhibit pyrexia and worsening altered sensorium with progressing renal failure, anemia, thrombocytopenia and respiratory failure. This constellation of symptoms caused the patient to require mechanical ventilation and hemodialysis. Upon laboratory analysis, hyperferritinemia provided an indication to the diagnosis of acquired hemophagocytic lymphohistiocytosis. After the initiation of dexamethasone, the patient made a significant recovery and was discharged from the hospital.
获得性噬血细胞性淋巴组织细胞增生症是一种罕见且致命的综合征，由免疫系统过度活跃引起，巨噬细胞和淋巴细胞不受控制地激活、细胞因子血症及全身炎症反应。一名75岁男性出现典型心绞痛，被诊断为急性冠状动脉综合征，需要进行经皮腔内冠状动脉成形术。然而症状并未缓解，患者开始出现发热，意识障碍加重，并伴有进行性肾衰竭、贫血、血小板减少和呼吸衰竭。这一系列症状导致患者需要机械通气和血液透析。实验室分析显示，高铁蛋白血症提示了获得性噬血细胞性淋巴组织细胞增生症的诊断。开始使用地塞米松治疗后，患者显著康复并出院。
