Vascular Surgery Department, Al-Razi Hospital, Aleppo, Syria.
Faculty of Medicine, University of Aleppo, Aleppo, Syria.
J Med Case Rep. 2024 Sep 10;18(1):423. doi: 10.1186/s13256-024-04760-4.
Hemophagocytic lymphohistiocytosis characterized by hemophagocytosis leading to uncontrolled inflammation; the most common etiology in secondary cases of hemophagocytic lymphohistiocytosis is viral infections, especially Epstein-Barr virus. Visceral leishmaniasis is a vectorborne protozoal disease caused by Leishmania donovani complex. It is common in tropical and subtropical regions, with 50,000-90,000 new cases annually.
A 15-month-old Arab female was admitted to our hospital with 15 days of fever and decreased weight. On clinical examination, she had a markedly enlarged liver and spleen that were palpable 4 cm and 6 cm below the costal margin, respectively. The peripheral blood smear showed hypochromic microcytic anemia, poikilocytosis, reactive lymphocytosis, and mild thrombocytopenia. Bone marrow aspiration did not show malignancy or any other pathological findings. The patient was put on antibiotic therapy without improvement. Repeated bone marrow aspiration showed erythrophagocytosis; intracellular small round organisms looked like the amastigote form of Leishmania (Donovan bodies) with no evidence of malignancies. Her lab values showed ferritin greater than 500 ug/L, pancytopenia, and hypertriglyceridemia. The patient was diagnosed with hemophagocytic lymphohistiocytosis secondary to visceral leishmaniasis.
Hemophagocytic lymphohistiocytosis secondary to visceral leishmaniasis is an extensively rare phenomenon in the medical literature that causes challenges in diagnosis and management. Steroids should be used wisely to not cover the symptoms of infections or malignancy, and amphotericin B resistance should be kept in mind in unresponsive Leishmania cases.
噬血细胞性淋巴组织细胞增生症的特征是发生吞噬作用,导致不受控制的炎症;噬血细胞性淋巴组织细胞增生症的继发性最常见病因是病毒感染,尤其是 EBV(Epstein-Barr virus)。内脏利什曼病是一种由杜氏利什曼原虫复合体引起的经媒介传播的原生动物病。它常见于热带和亚热带地区,每年有 5 万至 9 万例新发病例。
一名 15 个月大的阿拉伯女性因发热和体重减轻 15 天而入院。临床检查发现,她的肝脏和脾脏明显肿大,分别可在肋缘下触及 4 厘米和 6 厘米。外周血涂片显示低色素小细胞性贫血、异形红细胞、反应性淋巴细胞增多和轻度血小板减少。骨髓抽吸未显示恶性肿瘤或任何其他病理发现。患者接受抗生素治疗,但无改善。反复骨髓抽吸显示红细胞吞噬作用;细胞内的小圆体看起来像利什曼原虫的无鞭毛体形式(杜诺凡小体),没有恶性肿瘤的证据。她的实验室值显示铁蛋白大于 500ug/L、全血细胞减少和高甘油三酯血症。该患者被诊断为内脏利什曼病继发的噬血细胞性淋巴组织细胞增生症。
内脏利什曼病继发的噬血细胞性淋巴组织细胞增生症在医学文献中是一种罕见现象,这给诊断和管理带来了挑战。应谨慎使用类固醇,以免掩盖感染或恶性肿瘤的症状,并且在利什曼病治疗无反应的情况下应牢记两性霉素 B 耐药性。