Syed Salman, Shah Girish, Lachhar Garry, Patel Neel, Amjad Farhad Mohammad
Internal Medicine, Northwell Health, New York, USA.
Hematology and Oncology, Northwell Health, New York, USA.
Cureus. 2024 Oct 28;16(10):e72575. doi: 10.7759/cureus.72575. eCollection 2024 Oct.
Adult-onset hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by dysregulated immune activation. Diagnosing HLH poses significant challenges due to its nonspecific clinical presentation, which often mimics infections, malignancies, and autoimmune diseases. Early recognition and prompt initiation of immunosuppressive therapy are crucial, as delayed treatment is associated with a high risk of mortality. We present a unique case of HLH triggered by a combination of Epstein-Barr virus (EBV), Lyme disease, and anaplasmosis, underscoring the complexity of diagnosing and managing this condition. The patient initially presented with fever, hypotension, and altered mental status, raising concerns for septic shock. However, a comprehensive diagnostic workup, including infectious disease testing, hematologic evaluation, and bone marrow biopsy, revealed HLH with evidence of EBV viremia, exposure, and anaplasmosis infection. This case highlights the importance of maintaining a broad differential diagnosis when confronted with a sepsis-like presentation, particularly in endemic regions where tick-borne diseases and viral infections coexist. A multidisciplinary approach involving infectious disease specialists, hematologists, and intensivists was essential in achieving the diagnosis and formulating a treatment plan. The patient responded favorably to immunosuppressive therapy, including corticosteroids, intravenous immunoglobulins, and targeted antimicrobial therapy, resulting in clinical stabilization and recovery. Our report emphasizes the need for heightened clinical awareness of HLH and the challenges posed by its overlapping features with other systemic illnesses. Furthermore, it illustrates the significance of early intervention and individualized care in managing HLH triggered by multiple infectious agents.
成人起病的噬血细胞性淋巴组织细胞增生症（HLH）是一种危及生命的高炎症综合征，其特征为免疫激活失调。由于HLH的临床表现不具有特异性，常类似感染、恶性肿瘤和自身免疫性疾病，因此诊断HLH面临重大挑战。早期识别并迅速启动免疫抑制治疗至关重要，因为延迟治疗与高死亡风险相关。我们报告了一例由EB病毒（EBV）、莱姆病和无形体病共同引发的独特HLH病例，强调了诊断和管理该疾病的复杂性。患者最初表现为发热、低血压和精神状态改变，引发了对感染性休克的担忧。然而，包括传染病检测、血液学评估和骨髓活检在内的全面诊断检查显示为HLH，并伴有EBV病毒血症、莱姆病暴露和无形体病感染的证据。该病例突出了在面对类似脓毒症的表现时，尤其是在蜱传疾病和病毒感染并存的流行地区，保持广泛鉴别诊断的重要性。由传染病专家、血液学家和重症监护医生组成的多学科方法对于实现诊断和制定治疗计划至关重要。患者对免疫抑制治疗反应良好，包括使用皮质类固醇、静脉注射免疫球蛋白和针对性抗菌治疗，从而实现了临床稳定和康复。我们的报告强调了提高对HLH临床认识的必要性以及其与其他全身性疾病重叠特征所带来的挑战。此外，它还说明了在管理由多种感染因子引发的HLH时早期干预和个体化护理的重要性。
