PDF(Pubmed)
Abstract：
Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal complication of visceral leishmaniasis (VL). To provide a basis for early and correct diagnosis and to improve prognosis in the future, we describe a case series of VL-associated HLH in adults in our center in the past decade after review of all reported cases of adult VL-associated HLH in English through May 2022. In our case series, a total of 111 patients were diagnosed with VL. Among these patients, only six cases were diagnosed with VL-associated HLH. All patients tested positive for serology. Leishmania was detected for the first time by bone marrow aspiration (BMA) in three of the six patients and in the other three patients after three or four BMAs. It took more than 1 month from onset to diagnosis of VL for all the six cases, and the longest time was 6 months. Five of the six patients recovered after receiving sodium stibogluconate. VL-associated HLH is rare but potentially life-threatening in adults and predisposes to early delays in diagnosis. However, diagnostic techniques are not complicated or difficult, so it is more important to consider that it is not recognized by physicians. Although guidelines recommend liposomal amphotericin B as the most effective therapy, our experience suggests that sodium stibogluconate can be an alternative option when liposomal amphotericin B is unavailable or unaffordable.
摘要：
噬血细胞性淋巴组织细胞增生症（HLH）是内脏利什曼病（VL）的罕见致命并发症。为今后早期正确诊断和改善预后提供依据,我们在对截至2022年5月所有报告的成人VL相关HLH病例进行英文回顾后,描述了过去10年我们中心成人VL相关HLH的一系列病例.在我们的案例系列中，共有111例患者被诊断为VL。在这些患者中,只有6例诊断为VL相关HLH。所有患者血清学检测呈阳性。在6例患者中的3例和3例或4例BMA后的其他3例患者中，首次通过骨髓穿刺（BMA）检测到利什曼原虫。所有6例患者从发病到诊断为VL需要1个多月，最长的时间是6个月。六名患者中有五名在接受葡萄糖酸钠后康复。VL相关的HLH在成人中很少见，但可能危及生命，并且易于早期诊断延迟。然而,诊断技术并不复杂或困难，因此，更重要的是要考虑到它不被医生认可。尽管指南推荐脂质体两性霉素B作为最有效的治疗方法，我们的经验表明,当脂质体两性霉素B不可用或负担不起时,葡萄糖酸钠可以作为替代方案.
