Teaching Hospital, Peradeniya, Sri Lanka.
Department of Medicine, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka.
J Med Case Rep. 2023 Jun 1;17(1):224. doi: 10.1186/s13256-023-03967-1.
Haemophagocytic lymphohistiocytosis (HLH) is an uncommon systemic inflammatory syndrome that can happen secondary to numerous conditions. It rarely occurs due to dengue infection causing significant mortality and morbidity even with appropriate treatment. The outcome is further poor if the diagnosis of HLH is delayed or left untreated. Therefore, a high degree of clinical suspicion is paramount in diagnosing HLH.
A 17-year-old Sinhalese boy was admitted to a tertiary care hospital in Sri Lanka with a 4-day history of fever, headache, nausea, vomiting, and diarrhea. He was hemodynamically stable, and the serological investigation confirmed a dengue infection. On the fifth day of fever, he entered the critical phase of dengue infection, confirmed by ultrasound evidence of plasma leaking. However, he had ongoing high fever spikes during the critical phase, and even after the critical phase was over, the fever spikes continued. Simultaneously, hepatosplenomegaly was noticed, and he showed persistent thrombocytopenia, neutropenia, and anemia despite the resolution of the critical phase. Further, the workup revealed a serum ferritin level of > 3000 ng/mL triglyceride level of 314 mg/dL, and the bone marrow biopsy revealed an increased haemophagocytic activity. Secondary HLH was diagnosed on the basis of criteria used in the HLH-2004 trial and successfully managed with intravenous dexamethasone 10 mg/body surface area/day for the first 2 weeks, followed by a tapering regimen over 8 weeks.
This case emphasizes the need to consider HLH as a potential complication when persistent fever and cytopenias are present after recovering from dengue fever, particularly in patients with unusual clinical features like hepatosplenomegaly. Early recognition and prompt treatment with appropriate immunosuppressive therapy, such as intravenous dexamethasone, can lead to a successful response and good prognosis.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的全身性炎症综合征，可继发于多种疾病。即使接受适当的治疗，由登革热感染引起的 HLH 也会导致很高的死亡率和发病率。如果诊断延迟或未得到治疗，预后会更差。因此，高度的临床怀疑对于诊断 HLH 至关重要。
一名 17 岁的僧伽罗男孩因发热、头痛、恶心、呕吐和腹泻 4 天，入住斯里兰卡的一家三级保健医院。他血流动力学稳定，血清学检查证实存在登革热感染。发热的第 5 天，他进入登革热的危急期，超声检查证实有血浆渗出。然而，在危急期，他仍持续高热，甚至在危急期结束后，高热仍持续存在。同时，发现肝脾肿大，尽管危急期已过，但他仍持续出现血小板减少、中性粒细胞减少和贫血。此外，检查发现血清铁蛋白水平>3000ng/mL，甘油三酯水平 314mg/dL，骨髓活检显示噬血活性增加。根据 HLH-2004 试验中使用的标准，诊断为继发性 HLH，并成功地用静脉注射地塞米松 10mg/体表面积/天治疗 2 周，然后在 8 周内逐渐减量。
本病例强调，当从登革热恢复后出现持续发热和细胞减少时，特别是在有肝脾肿大等不典型临床表现的患者中，应考虑 HLH 作为潜在并发症。早期识别并及时给予适当的免疫抑制治疗，如静脉注射地塞米松，可以获得良好的反应和预后。
