BACKGROUND. 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%. OBJECTIVE. To raise awareness of HLH among healthcare professionals, particularly intensivists. METHODS. We report nine cases of secondary HLH seen at tertiary hospitals in Bloemfontein, South Africa. RESULTS. 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. CONCLUSION. A high index of suspicion may promote the accurate diagnosis of HLH in patients presenting with fever, transaminitis and unexplained cytopenia.
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机译：背景。血症细胞淋巴管激瘤症（HLH）是一个潜在的危及生命危及危及综合征，如果没有早期认可和管理。它涉及免疫系统的不受控制的病理激活，它是遗传或获得的。它呈现出严重炎症的临床和实验室特征。早期开始有效治疗可能会降低95％至35％的死亡率。客观的。提高医疗专业人士，特别是强烈主义者的HLH的认识。方法。我们报告了南非Bloemfontein的第三届医院见面的九次次HLH病例。结果。所有患者患有发烧，高甘油病症，高温血症，蛋白质炎和细胞凋亡。在患者的66.7％（n = 6/9）骨髓活检上注意到血症细胞增多症。淋巴瘤引发了超过三分之一的病例（44.4％; n = 4/9），44％（n = 4/9）与感染有关，11％（n = 1/9）相关HIV 。最后，患者的11.1％（n = 1）被潜在的自身免疫疾病引发。案件的一半以上（55.6％; n = 5/9）有致命结果。结论。高度的怀疑指标可以促进患有发烧，越野炎和未解释的细胞贫症的患者中的HLH的准确诊断。
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