Jasmine Y S Y, Lee S L, Kan F K
Hospital Pakar Sultanah Fatimah, Medical Department, Muar, Johor, Malaysia.
Hospital Sultanah Aminah, Internal Medicine (Infectious Diseases), Johor Bahru, Johor, Malaysia.
Med J Malaysia. 2017 Feb;72(1):62-64.
Haemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disorder resulting from uncontrolled hyperinflammatory response. There had been increase in cases of one of the secondary form of HLH, i.e., infectionassociated haemophagocytic syndrome (IAHS) in severe dengue in recent years. However, the condition remains under diagnosed due to lack of awareness compounded by the lack of validated diagnostic criteria. Severe hepatitis with prolonged cytopenias, severe hyperferritinemia, hypofibrinogenemia and persistent fever were evident in all four cases reported. All the subjects survived with supportive care and adjuvant steroid therapy. Prospective controlled studies are needed to develop diagnostic criteria and management protocol for IAHS in severe dengue.
噬血细胞性淋巴组织细胞增生症(HLH)是一种由不受控制的过度炎症反应导致的潜在致命性疾病。近年来,严重登革热中HLH的一种继发形式,即感染相关噬血细胞综合征(IAHS)的病例有所增加。然而,由于认识不足以及缺乏经过验证的诊断标准,该病仍未得到充分诊断。在报告的所有4例病例中,均出现了伴有长期血细胞减少的严重肝炎、严重高铁蛋白血症、低纤维蛋白原血症和持续发热。所有患者通过支持治疗和辅助性类固醇治疗存活下来。需要进行前瞻性对照研究,以制定严重登革热中IAHS的诊断标准和管理方案。