背景:噬血细胞性淋巴组织细胞增生症(HLH)是一种具有潜在致命后果的综合征,其由功能失调的自然杀伤细胞和细胞毒性T淋巴细胞引起的过度免疫反应。继发性HLH,这是成年人的主要类型,与各种医疗状况有关,包括感染,恶性肿瘤,和自身免疫性疾病。与中暑相关的继发性HLH尚未报告。
方法:一名74岁男性在42°C的热水公共浴池中失去知觉后进入急诊科。患者被目睹在水中超过4小时。患者病情并发横纹肌溶解和脓毒性休克,用机械通气管理,血管活性剂,和连续性肾脏替代疗法。患者还表现出弥漫性脑功能障碍的证据。
方法:当患者的病情初步好转时,病人发烧了,贫血,血小板减少症,总胆红素急性升高,which,我们怀疑,是由HLH引起的。进一步的研究显示血清铁蛋白和可溶性白介素2受体水平升高。
方法:患者接受2个周期的连续治疗性血浆置换以降低内毒素负荷。为了管理HLH,大剂量糖皮质激素治疗.
结果:尽管尽了最大努力,患者未从进行性肝功能衰竭中康复并过期.
结论:我们报告了一例继发HLH与中暑相关的新病例。诊断继发性HLH可能很困难,因为潜在疾病和HLH的临床表现可能同时存在。需要早期诊断和及时开始治疗以改善疾病的预后。
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome with potentially fatal consequences that results from an excessive immune response caused by malfunctioning natural killer cells and cytotoxic T lymphocytes. Secondary HLH, which is the predominant type in adults, is associated with various medical conditions, including infections, malignancies, and autoimmune diseases. Secondary HLH associated with heat stroke has not been reported.
METHODS: A 74-year-old male was admitted to the emergency department after being unconscious in a 42°C hot public bath. The patient was witnessed to be in the water for more than 4 hours. The patient\'s condition was complicated by rhabdomyolysis and septic shock, which were managed with mechanical ventilation, vasoactive agents, and continuous renal replacement therapy. The patient also showed evidence of diffuse cerebral dysfunction.
METHODS: While the patient\'s condition initially improved, the patient developed a fever, anemia, thrombocytopenia, and an acute rise in total bilirubin, which, we suspected, was caused by HLH. Further investigations revealed elevated serum ferritin and soluble interleukin-2 receptor levels.
METHODS: The patient received 2 cycles of serial therapeutic plasma exchange to lower the endotoxin burden. To manage HLH, high-dose glucocorticoid therapy was done.
RESULTS: Despite the best efforts, the patient did not recover and expired from progressive hepatic failure.
CONCLUSIONS: We report a novel
case of secondary HLH associated with heat stroke. Diagnosing secondary HLH can be difficult since clinical manifestations of the underlying disease and HLH may present simultaneously. Early diagnosis and prompt initiation of treatment is required to improve the prognosis of the disease.