登革热相关的噬血细胞淋巴组织细胞病伴严重多器官并发症1例报告

R. Agrawal, P. Agrawal, Sudha Madhuri, Thakur Abhijit Singh
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引用次数: 1

摘要

噬血细胞性淋巴组织细胞增多症(HLH)是一种罕见的危及生命的综合征,其特征是病理性免疫过度激活、极端炎症和多器官受累并伴有可变细胞减少。我们报告一例53岁男性,无已知合并症,表现为持续的登革热、全血细胞减少症、高铁蛋白水平和多器官功能障碍,尽管接受了治疗,但临床进展恶化。根据临床实验室检查结果,他在入院第6天被诊断为登革热相关的HLH。根据HLH治疗方案,他开始使用免疫抑制剂并显示出显着改善。在病情严重的登革热患者中,住院时间延长、持续细胞减少和多器官功能障碍与血浆漏相不相称,应引起高度怀疑感染引发的HLH的可能性。虽然及时诊断和早期治疗是防止不可逆终末器官损伤和死亡的关键,但障碍在于其在成人中罕见,临床表现多变,实验室结果非特异性。
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Dengue-Associated Hemophagocytic Lymphohistiocytosis With Severe Multiorgan Complications—A Case Report
Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening syndrome characterized by pathologic immune hyperactivation, extreme inflammation, and multiorgan involvement with variable cytopenias. We report a case of a 53-year-old male with no known comorbidities, who presented with unrelenting dengue fever, pancytopenia, high ferritin levels, and multiple organ dysfunction with progressive clinical deterioration despite treatment. Based on the clinicolaboratory findings, he was diagnosed as dengue-associated HLH on day 6 of admission. As per HLH treatment protocol, he was started on immunosuppressants and showed significant improvement. In dengue patients with severe disease, prolonged hospital stay, persistent cytopenia, and multiorgan dysfunction out of proportion to the plasma leakage phase should prompt a high index of suspicion for the possibility of infection-triggered HLH. Although prompt diagnosis and early initiation of therapy is the key to prevent irreversible end organ damage and mortality, the hindrance lies in its rarity in adults, variable clinical presentation, and nonspecific laboratory findings.
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期刊介绍: Indian Association of Clinical Medicine is an academic body constituted in the year 1992 by a group of clinicians with the main aim of reaffirming the importance of clinical medicine in this era of high-tech diagnostic modalities. There is no doubt that modern investigational methods have contributed a lot to the present day medical practice but that does not render clinical acumen and examination less important. The art and science of clinical medicine helps up to make proper and judicious use of investigations and not these be the sole basis of our practice. That is the basic idea behind this ''Association''. We presently have members and fellows of the association from all over the country. In August, 2002 the body was registered as "Indian Association of Clinical Medicine" by the Registrar of Societies, Delhi.
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