Arora Amodini, Verma Sarita, Khot Nikita, Chalipat Shiji, Agarkhedkar Sharad, Kiruthiga Kala Gnanasekaran
Pediatrics, Dr. D.Y. Patil Medical College, Pune, IND.
Pediatric Oncology, KEM Hospital & Research Centre, Pune, IND.
Cureus. 2023 Feb 8;15(2):e34773. doi: 10.7759/cureus.34773. eCollection 2023 Feb.
India is an endemic country for dengue. The incidence of hemophagocytic lymphohistiocytosis (HLH) with dengue in children has been well-reported. However, central nervous system (CNS) HLH associated with dengue has not been described in the literature yet. We hereby report a novel case of CNS HLH triggered by dengue infection. An eight-month-old, well-grown male infant with uneventful antenatal, perinatal, and neonatal history was admitted with a history of febrile illness associated with cough, cold, vomiting, and loose motions and one episode of hematochezia and hepatosplenomegaly on examination. Investigations revealed bi-cytopenia, hyper-ferritinemia, deranged coagulation profile, liver function test, and hypo-fibrinogenemia. Dengue non-structural protein 1 ( NS1) antigen was positive. The child was given dexamethasone and continued supportive care with a diagnosis of dengue shock syndrome. The child showed an overall transient improvement, however, he had rebound fever followed by right focal convulsion on Day 9 of steroids. MRI brain revealed areas of diffusion-restricted embolic infarcts with diffuse leptomeningeal enhancement and mild cerebral edema, and CSF showed a total leukocyte count of 80 cells with 75% lymphocytic picture, histiocytes with hemophagocytosis, confirmatory of CNS HLH. Intrathecal methotrexate, hydrocortisone, and intravenous (IV) etoposide were started. However, the child succumbed to his illness. CNS involvement in dengue-triggered HLH needs to be suspected despite subtle neurological signs and aggressively managed following a multi-departmental approach to ensure the best clinical and neuro-developmental outcomes.
印度是登革热的流行国家。儿童登革热合并噬血细胞性淋巴组织细胞增生症（HLH）的发病率已有充分报道。然而，文献中尚未描述与登革热相关的中枢神经系统（CNS）HLH。我们在此报告一例由登革热感染引发的CNS HLH新病例。一名8个月大、生长发育良好的男婴，产前、围产期及新生儿期均无异常，因发热性疾病伴咳嗽、感冒、呕吐、腹泻病史入院，检查发现有一次便血及肝脾肿大。检查发现双血细胞减少、高铁蛋白血症、凝血功能紊乱、肝功能检查异常及低纤维蛋白原血症。登革热非结构蛋白1（NS1）抗原呈阳性。该患儿被诊断为登革热休克综合征，给予地塞米松并继续进行支持治疗。患儿总体上有短暂改善，但在使用类固醇第9天出现反复发热，随后出现右侧局灶性惊厥。脑部MRI显示弥散受限的栓塞性梗死区域，伴有弥漫性软脑膜强化和轻度脑水肿，脑脊液显示白细胞总数为80个细胞，淋巴细胞占75%，有噬血细胞的组织细胞，确诊为CNS HLH。开始鞘内注射甲氨蝶呤、氢化可的松及静脉注射依托泊苷。然而，患儿最终因病死亡。尽管神经系统体征不明显，但仍需怀疑登革热引发的HLH累及中枢神经系统，并应采取多部门积极管理措施，以确保最佳的临床和神经发育结局。
