Atim-Oluk M
Hull and East Yorkshire National Health Service Trust, Castle Hill Hospital Castle Road, Cottingham, East Yorkshire, HU16 5JQ United Kingdom.
Eur J Microbiol Immunol (Bp). 2013 Mar;3(1):81-9. doi: 10.1556/EuJMI.3.2013.1.12. Epub 2013 Mar 13.
Haemophagocytic lymphohistiocytosis (HLH) describes a rare, poorly recognised and under-diagnosed immunopathological syndrome whereby there is a highly stimulated yet ineffective multisystem inflammatory response [1]. I present the first case in English literature of Cytomegalovirus (CMV) associated HLH diagnosed by clinical and serological means, and the fourth case of CMV associated haemophagocytic lymphohistiocytosis in an immunocompetent adult, according to HLH-2004 diagnostic guidelines. I include a literature review of CMV associated HLH in adults and raise awareness of checking serum ferritin in patients who present with a sepsis like syndrome. Additionally, this article discusses the merits of HLH-2004 diagnostic work-up without bone marrow biopsy, using clinical and serological means only. I support the reclassification of HLH alongside the other hyperinflammatory syndromes of SIRS, sepsis, septic shock, and MODS to improve understanding and recognition.
噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见、认识不足且诊断不足的免疫病理综合征,其特征是存在高度激活但无效的多系统炎症反应[1]。根据HLH - 2004诊断指南,我报告了英文文献中首例通过临床和血清学手段诊断的巨细胞病毒(CMV)相关HLH病例,也是免疫功能正常成年人中的第四例CMV相关噬血细胞性淋巴组织细胞增生症病例。我对成人CMV相关HLH进行了文献综述,并提高了对出现脓毒症样综合征患者检查血清铁蛋白的认识。此外,本文讨论了仅使用临床和血清学手段、无需骨髓活检的HLH - 2004诊断检查的优点。我支持将HLH与全身炎症反应综合征(SIRS)、脓毒症、脓毒性休克和多器官功能障碍综合征(MODS)等其他过度炎症综合征一起重新分类,以增进理解和识别。