PDF(Pubmed)
Abstract：
UNASSIGNED: Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome if not recognised and managed early. It involves an uncontrolled pathological activation of the immune system, and it is either genetic or acquired. It presents with clinical and laboratory features of severe inflammation. Early initiation of effective therapy may reduce mortality from 95% to 35%.
UNASSIGNED: To raise awareness of HLH among healthcare professionals, particularly intensivists.
UNASSIGNED: We report nine cases of secondary HLH seen at tertiary hospitals in Bloemfontein, South Africa.
UNASSIGNED: All patients presented with fever, hypertriglyceridaemia, hyperferritinaemia, transaminitis and cytopenia. Haemophagocytosis was noted on bone marrow biopsy in 66.7% (n=6/9) of the patients. More than one-third (44.4%; n=4/9) of the cases were triggered by a lymphoma, 44% (n=4/9) were associated with infection and 11% (n=1/9) were associated HIV. Finally, 11.1% (n=1) of the patients were triggered by an underlying autoimmune disease. More than half (55.6%; n=5/9) of the cases had a fatal outcome.
UNASSIGNED: A high index of suspicion may promote the accurate diagnosis of HLH in patients presenting with fever, transaminitis and unexplained cytopenia.
UNASSIGNED: HLH is a rare, life-threatening condition that may be missed in the intensive care setting. This report emphasises the importance of clinical suspicion, early diagnosis and appropriate intervention.
UNASSIGNED: To raise awareness of HLH among healthcare professionals, particularly intensivists.
UNASSIGNED: We report nine cases of secondary HLH seen at tertiary hospitals in Bloemfontein, South Africa.
UNASSIGNED: All patients presented with fever, hypertriglyceridaemia, hyperferritinaemia, transaminitis and cytopenia. Haemophagocytosis was noted on bone marrow biopsy in 66.7% (n=6/9) of the patients. More than one-third (44.4%; n=4/9) of the cases were triggered by a lymphoma, 44% (n=4/9) were associated with infection and 11% (n=1/9) were associated HIV. Finally, 11.1% (n=1) of the patients were triggered by an underlying autoimmune disease. More than half (55.6%; n=5/9) of the cases had a fatal outcome.
UNASSIGNED: A high index of suspicion may promote the accurate diagnosis of HLH in patients presenting with fever, transaminitis and unexplained cytopenia.
UNASSIGNED: HLH is a rare, life-threatening condition that may be missed in the intensive care setting. This report emphasises the importance of clinical suspicion, early diagnosis and appropriate intervention.
摘要：
未经证实：噬血细胞性淋巴组织细胞增生症（HLH）是一种潜在的威胁生命的综合征。它涉及免疫系统不受控制的病理激活，它要么是遗传的，要么是后天的。它具有严重炎症的临床和实验室特征。早期开始有效治疗可以将死亡率从95%降低到35%。
未经批准：为了提高医疗保健专业人员对HLH的认识，尤其是强化主义者。
未经证实：我们报告了布隆方丹三级医院的9例继发性HLH，南非。
未经证实：所有患者均出现发热，高甘油三酯血症，高铁蛋白血症,转氨酶和血细胞减少症。66.7％（n=6/9）的患者在骨髓活检中发现了吞噬作用。超过三分之一（44.4%；n=4/9）的病例是由淋巴瘤引发的，44%（n=4/9）与感染相关，11%（n=1/9）与HIV相关。最后,11.1％（n=1）的患者是由潜在的自身免疫性疾病引发的。超过一半（55.6％；n=5/9）的病例具有致命结局。
未经证实：高怀疑指数可能会促进发热患者HLH的准确诊断，转氨酶和无法解释的血细胞减少症。
未经证实：HLH是一种罕见的，在重症监护环境中可能会错过的危及生命的疾病。这份报告强调了临床怀疑的重要性，早期诊断和适当干预。
未经批准：为了提高医疗保健专业人员对HLH的认识，尤其是强化主义者。
未经证实：我们报告了布隆方丹三级医院的9例继发性HLH，南非。
未经证实：所有患者均出现发热，高甘油三酯血症，高铁蛋白血症,转氨酶和血细胞减少症。66.7％（n=6/9）的患者在骨髓活检中发现了吞噬作用。超过三分之一（44.4%；n=4/9）的病例是由淋巴瘤引发的，44%（n=4/9）与感染相关，11%（n=1/9）与HIV相关。最后,11.1％（n=1）的患者是由潜在的自身免疫性疾病引发的。超过一半（55.6％；n=5/9）的病例具有致命结局。
未经证实：高怀疑指数可能会促进发热患者HLH的准确诊断，转氨酶和无法解释的血细胞减少症。
未经证实：HLH是一种罕见的，在重症监护环境中可能会错过的危及生命的疾病。这份报告强调了临床怀疑的重要性，早期诊断和适当干预。
