Diamantidis Michael D, Palioura Andromachi, Ioannou Maria, Tsangalas Evangelos, Karakousis Konstantinos
Hematology, First Department of Internal Medicine, Thalassemia and Sickle Cell Disease Unit, General Hospital of Larissa, Larissa, GRC.
Internal Medicine, First Department of Internal Medicine, General Hospital of Larissa, Larissa, GRC.
Cureus. 2020 Dec 4;12(12):e11911. doi: 10.7759/cureus.11911.
Hemophagocytic lymphohistiocytosis (HLH), or hemophagocytic syndrome (HS) is a severe syndrome involving an extreme participation of the immune system, resulting in a cascade of cytokines, hyperinflammation and extensive hemophagocytosis in the bone marrow (BM) and affecting the peripheral blood (PB) lineages. Fever, splenomegaly, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia are often encountered in this disease. The syndrome can be seen in all ages and it is either primary due to genetic defects or secondary because of malignancies, immune deficiencies, rheumatic diseases, and infections. Bacteria, viruses, protozoa, and fungi are often implicated. Visceral leishmaniasis (VL) is among the infectious causes of HLH. We describe a patient with a successful treatment of HLH after the initiation of liposomal amphotericin B, due to VL, even though there was a delay in diagnosing the leishmaniasis. The exact precipitating pathophysiological events triggering HLH remain unknown and provide their clear impact for future research. An instructive, critical review of the literature related to the presented case is provided. Distinguishing secondary HS from primary HS is essential for the application of suitable treatment. Improper use of corticosteroids could cover up an underlying possible malignancy or infection and delay the initiation of the etiologic therapeutic strategy.
噬血细胞性淋巴组织细胞增生症（HLH），或噬血细胞综合征（HS）是一种严重的综合征，涉及免疫系统的过度参与，导致细胞因子级联反应、炎症反应亢进以及骨髓（BM）中广泛的噬血细胞现象，并影响外周血（PB）谱系。发热、脾肿大、高甘油三酯血症、低纤维蛋白原血症和高铁蛋白血症在该疾病中较为常见。该综合征可见于各年龄段，可为原发性，由基因缺陷引起，也可为继发性，继发于恶性肿瘤、免疫缺陷、风湿性疾病和感染。细菌、病毒、原生动物和真菌常与之相关。内脏利什曼病（VL）是HLH的感染性病因之一。我们描述了一名因VL导致HLH的患者，尽管利什曼病的诊断存在延迟，但在开始使用脂质体两性霉素B治疗后成功治愈了HLH。引发HLH的确切病理生理事件仍不清楚，这为未来的研究提供了明确的方向。本文对与该病例相关的文献进行了有指导意义的批判性综述。区分继发性HS和原发性HS对于应用合适的治疗方法至关重要。不恰当使用糖皮质激素可能掩盖潜在的恶性肿瘤或感染，并延迟病因治疗策略的启动。
