Division of Pediatric Hematology and Oncology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.
Pediatr Blood Cancer. 2010 Oct;55(4):754-6. doi: 10.1002/pbc.22618.
Secondary hemophagocytic lymphohistiocytosis (sHLH) is a reactive, proliferative disorder of the immune system resulting in lymphohistiocytic proliferation, hemophagocytosis, and cytokine dysregulation. The most common infectious trigger in sHLH is Epstein-Barr virus (EBV-HLH). Current treatment protocols for EBV-HLH have a cure rate of approximately 75%; however, there are significant toxicities associated with these therapies. We present two patients with EBV-HLH who experienced spontaneous resolution of their disease prior to the initiation of therapy, suggesting there may be a subgroup of patients with EBV-HLH who do well with conservative management and can avoid potentially toxic therapies.
继发性噬血细胞性淋巴组织细胞增生症(sHLH)是一种免疫反应性、增生性疾病,导致淋巴组织细胞增生、噬血细胞和细胞因子失调。sHLH 最常见的感染触发因素是 EBV(EBV-HLH)。目前 EBV-HLH 的治疗方案的治愈率约为 75%;然而,这些治疗方法存在显著的毒性。我们报告了 2 例 EBV-HLH 患者,他们在开始治疗前疾病自发缓解,这表明可能存在一小部分 EBV-HLH 患者采用保守治疗效果良好,可以避免潜在的毒性治疗。