Department of Hematology, The First Affiliated Hospital and College of Clinical Medicine, Henan University of Science and Technology, Luoyang, China.
College of Medicine and Public Health, Flinders University, Adelaide, Australia.
Am J Trop Med Hyg. 2023 Jul 10;109(2):296-300. doi: 10.4269/ajtmh.22-0776. Print 2023 Aug 2.
Visceral leishmaniasis-related hemophagocytic lymphohistiocytosis (VL-HLH) is a potentially life-threatening secondary hemophagocytic lymphocytic syndrome caused by protozoan parasites of the Leishmania species and transmitted by infected sandflies. Therefore, it is important to be highly vigilant of the infection, particularly the visceral subtype, to share information with the public health system, and to improve the early diagnosis rate so that appropriate treatment can be initiated promptly. We report two isolated cases of VL-HLH. The main clinical manifestations were fever, pancytopenia, splenomegaly, hypofibrinogenemia, and hyperferremia, which meet the HLH-2004 diagnostic criteria. In our experience, anti-HLH treatment was not very effective for either case. No Leishmania organism was found in the first bone marrow smear of either patient. The first patient was diagnosed after identification of Leishmania amastigotes via sternal bone marrow biopsy, rK39 immunochromatography test, and metagenomic next-generation sequencing. The other patient was diagnosed by rK39-rapid diagnostic test and polymerase chain reaction. However, because of the delayed diagnosis in both cases, their conditions continued to deteriorate and both patients eventually died of the disease. Leishmaniasis is a parasitic disease with regional specificity and a low incidence. The occurrence of secondary HLH has a great impact on prognosis. When encountering secondary HLH in clinical practice, leishmaniasis should remain on the list of differential causes. Because of a high mortality rate if diagnosed late, it is crucial to be vigilant of VL-HLH in practice so that early detection, diagnosis, and treatment of the disease can be achieved to reduce adverse patient outcomes.
内脏利什曼病相关噬血细胞性淋巴组织细胞增生症（VL-HLH）是一种潜在危及生命的继发性噬血细胞性淋巴组织细胞增生综合征，由利什曼原虫属的原生动物寄生虫引起，通过受感染的沙蝇传播。因此，高度警惕感染非常重要，特别是内脏利什曼病，与公共卫生系统共享信息，并提高早期诊断率，以便及时启动适当的治疗。我们报告了两例孤立的 VL-HLH 病例。主要临床表现为发热、全血细胞减少、脾肿大、低纤维蛋白原血症和高血铁症，符合 HLH-2004 诊断标准。根据我们的经验，抗 HLH 治疗对这两个病例都不是很有效。两个患者的第一次骨髓涂片均未发现利什曼原虫。第一个患者在胸骨骨髓活检、rK39 免疫层析试验和宏基因组下一代测序鉴定出利什曼无鞭毛体后被诊断。另一个患者通过 rK39-快速诊断试验和聚合酶链反应被诊断。然而，由于两个病例的诊断均延迟，他们的病情持续恶化，最终两名患者均因该病死亡。利什曼病是一种具有区域特异性和低发病率的寄生虫病。继发性 HLH 的发生对预后有很大影响。当临床上遇到继发性 HLH 时，应将利什曼病保留在鉴别诊断的列表中。由于诊断延迟的死亡率很高，因此在实践中必须警惕 VL-HLH，以便早期发现、诊断和治疗疾病，以降低不良患者结局的风险。
