Epstein-Barr Virus encephalitis associated hemophagocytic lymphohistiocytosis in childhood-onset systemic lupus erythematosus: a case-based review.

IF 2.8 3区 医学 Q1 PEDIATRICS Pediatric Rheumatology Pub Date : 2024-10-31 DOI:10.1186/s12969-024-01025-8
Krit Cheawcharnpraparn, Thiraporn Kanjanaphan, Oranooj Lertkovit, Napaporn Puripat, Chutima Chavanisakun, Ornatcha Sirimongkolchaiyakul, Sirikarn Tangcheewinsirikul
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Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is characterized by immune dysregulation that results in an uncontrolled hyperinflammatory state. HLH is classified into two main categories: primary (familial) HLH and secondary (acquired) HLH. Secondary HLH can result from various underlying, including infection-associated hemophagocytic syndrome (IAHS) and macrophage activation syndrome (MAS) associated with rheumatologic disorders, among others. Epstein-Barr virus (EBV) often causes IAHS, but central nervous system (CNS) involvement is rare among systemic lupus erythematosus (SLE) patients. We report a case of EBV encephalitis associated with HLH in a patient with childhood-onset SLE.

Case presentation: A 12-year-old girl had received a diagnosis of SLE 2 months before presentation. After a period of inactive disease on treatment, fever and seizures, with altered mental status and hallucinations, developed over several weeks. A complete blood cell count (CBC) revealed pancytopenia, accompanied by elevated levels of inflammatory markers: 86 mm/hr erythrocyte sedimentation rate, 8.9 mg/dl c-reactive protein, and 3,966 ng/mL of ferritin. The differential diagnosis included active neuropsychiatric SLE, CNS infection and neurological manifestations in secondary HLH, which could have represented either IAHS or MAS. Meropenem and acyclovir were initially administered for clinical acute encephalitis, followed by pulse methylprednisolone; however, the fever persisted, and another CBC revealed progressive cytopenia. A bone marrow study showed hypocellularity and active hemophagocytic activity, and intravenous immunoglobulin was additionally given due to the diagnosis of HLH. Cerebrospinal fluid (CSF) analysis showed 60/mm3 white blood cells (N 55%, L 45%), 141 mg/dL glucose (0.7 blood-CSF glucose ratio), < 4 mg/dL protein; results of Gram stain and bacterial culture were negative. The viral encephalitis panel from the CSF confirmed EBV infection. Bone marrow immunohistochemistry examination revealed increasing levels of CD8 + T-cell and equivocal positive results for EBV-encoded RNA in situ hybridization; therefore, HLH potentially associated with EBV was diagnosed. After treatment with IVIg, cyclosporin A, and prednisolone, the patient's symptoms gradually improved and she was eventually able to return to school.

Conclusions: Our case highlights the importance of a thorough differential diagnosis, including EBV encephalitis associated with HLH, in patients with childhood SLE, particularly in cases of clinical deterioration occurs after initial treatment.

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儿童期系统性红斑狼疮中与嗜血细胞淋巴组织细胞增多症相关的爱泼斯坦-巴氏病毒脑炎:基于病例的综述。
背景:嗜血细胞淋巴组织细胞增多症(HLH嗜血细胞淋巴组织细胞增多症(HLH)的特点是免疫失调,导致无法控制的高炎症状态。嗜血细胞性淋巴细胞增多症分为两大类:原发性(家族性)嗜血细胞性淋巴细胞增多症和继发性(获得性)嗜血细胞性淋巴细胞增多症。继发性 HLH 可由多种原因引起,包括感染相关性嗜血细胞综合征(IAHS)和与风湿性疾病相关的巨噬细胞活化综合征(MAS)等。爱泼斯坦-巴氏病毒(EBV)通常会导致嗜血细胞增多症,但在系统性红斑狼疮(SLE)患者中,中枢神经系统(CNS)受累的情况并不多见。我们报告了一例儿童期系统性红斑狼疮患者的EB病毒脑炎伴HLH病例:病例介绍:一名12岁的女孩在就诊前2个月被诊断为系统性红斑狼疮。经过一段时间的治疗后,她的病情并无好转,但在数周内出现了发热和癫痫发作,并伴有精神状态改变和幻觉。全血细胞计数(CBC)显示为全血细胞减少,伴有炎症标志物水平升高:红细胞沉降率为 86 毫米/小时,c 反应蛋白为 8.9 毫克/分升,铁蛋白为 3,966 纳克/毫升。鉴别诊断包括活动性神经精神系统性红斑狼疮、中枢神经系统感染和继发性HLH的神经系统表现,这可能代表IAHS或MAS。起初,医生针对临床急性脑炎使用了美罗培南和阿昔洛韦,随后又使用了脉冲甲基强的松龙;然而,发热持续不退,再次进行全血细胞计数检查发现,患者的全血细胞计数呈进行性下降。骨髓检查显示细胞功能减退,嗜血细胞活性活跃,由于诊断为 HLH,因此又静脉注射了免疫球蛋白。脑脊液(CSF)分析显示白细胞为 60 个/立方毫米(N 55%,L 45%),葡萄糖为 141 毫克/分升(血液-脑脊液葡萄糖比值为 0.7):我们的病例强调了对儿童系统性红斑狼疮患者进行全面鉴别诊断的重要性,包括与 HLH 相关的 EBV 脑炎,尤其是在最初治疗后临床症状恶化的病例中。
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来源期刊
Pediatric Rheumatology
Pediatric Rheumatology PEDIATRICS-RHEUMATOLOGY
CiteScore
4.10
自引率
8.00%
发文量
95
审稿时长
>12 weeks
期刊介绍: Pediatric Rheumatology is an open access, peer-reviewed, online journal encompassing all aspects of clinical and basic research related to pediatric rheumatology and allied subjects. The journal’s scope of diseases and syndromes include musculoskeletal pain syndromes, rheumatic fever and post-streptococcal syndromes, juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, local and systemic scleroderma, Kawasaki disease, Henoch-Schonlein purpura and other vasculitides, sarcoidosis, inherited musculoskeletal syndromes, autoinflammatory syndromes, and others.
期刊最新文献
The patient's voice: a cross-sectional study of physical health and disability in juvenile idiopathic arthritis. Pediatric case of immune-mediated necrotizing myopathy with anti-HMGCR antibodies and dermatomyositis skin rash. Epstein-Barr Virus encephalitis associated hemophagocytic lymphohistiocytosis in childhood-onset systemic lupus erythematosus: a case-based review. Correction: Development and validation of a pediatric internationally agreed ultrasound knee synovitis protocol (PIUS-knee) by the PReS imaging working party. Clinical characteristics and prognosis of interstitial lung disease in systemic juvenile idiopathic arthritis: a two-center retrospective observational cohort study.
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