Center of Infectious Diseases, West China Hospital, Sichuan University, Chengdu, China.
Department of Hematology, West China Hospital, Sichuan University, Chengdu, China.
Am J Trop Med Hyg. 2022 Nov 14;107(6):1203-1209. doi: 10.4269/ajtmh.22-0361. Print 2022 Dec 14.
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.
噬血细胞性淋巴组织细胞增生症（HLH）是内脏利什曼病（VL）罕见且致命的并发症。为了提供早期和正确诊断的依据，并提高未来的预后，我们回顾了截至 2022 年 5 月在英语文献中报道的所有成人 VL 相关 HLH 病例，并在此基础上描述了我们中心过去十年中成人 VL 相关 HLH 的病例系列。在我们的病例系列中，共诊断出 111 例 VL 患者。在这些患者中，只有 6 例被诊断为 VL 相关 HLH。所有患者的血清学检测均呈阳性。6 例患者中的 3 例首次通过骨髓抽吸（BMA）检测到利什曼原虫，另外 3 例在 3 或 4 次 BMA 后检测到。所有 6 例患者从发病到诊断 VL 的时间均超过 1 个月，最长时间为 6 个月。6 例患者中有 5 例在接受葡萄糖酸锑钠治疗后痊愈。VL 相关 HLH 在成人中罕见但有潜在致命性，且易导致早期诊断延迟。然而，诊断技术并不复杂或困难，因此更重要的是要考虑到医生没有认识到这种疾病。尽管指南推荐两性霉素 B 脂质体作为最有效的治疗方法，但我们的经验表明，在无法获得或无法负担两性霉素 B 脂质体时，葡萄糖酸锑钠可以作为替代选择。
