Abstract：
Hydroa vacciniforme lymphoproliferative disorder (HV-LPD) and severe mosquito bite allergy (SMBA) are both cutaneous forms of Epstein-Barr virus (EBV)-associated T/natural killer (NK) cell LPDs and are closely related to chronic active EBV disease (CAEBV) and EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH). HV-LPD is further divided into classic HV, a benign subtype mediated by EBV-positive γδT cells, and systemic HV, another life-threatening subtype mainly associated with EBV-positive αβT or γδT cells. The vast majority of patients with SMBA have increased numbers of EBV-infected NK cells in the blood. Clinical symptoms of HV-LPD and SMBA often overlap in the same patient and may progress to more serious disease conditions equivalent to the systemic form of CAEBV. To define the disease spectrum of HV-LPD and SMBA, we propose the diagnostic criteria and the determination criteria for disease severity. The proposed diagnostic criteria are consistent with those for CAEBV and EBV-HLH in the guidelines for the management for CAEBV and related disorders 2023.
摘要：
水生疫苗淋巴增生性疾病（HV-LPD）和严重的蚊虫叮咬过敏（SMBA）都是爱泼斯坦-巴尔病毒（EBV）相关的T/自然杀伤（NK）细胞LPDs的皮肤形式，并且与慢性活动性EBV疾病（CAEBV）和EBV相关的噬血细胞淋巴组织细胞增多症（EBV-HLH）密切相关。HV-LPD进一步分为经典HV，由EBV阳性γδT细胞介导的良性亚型，和系统性HV，另一种威胁生命的亚型主要与EBV阳性αβT或γδT细胞有关。绝大多数SMBA患者血液中EBV感染的NK细胞数量增加。HV-LPD和SMBA的临床症状通常在同一患者中重叠，并且可能发展为与CAEBV的全身形式相当的更严重的疾病状况。为了定义HV-LPD和SMBA的疾病谱，我们提出了疾病严重程度的诊断标准和判定标准。拟议的诊断标准与2023年CAEBV和相关疾病治疗指南中CAEBV和EBV-HLH的诊断标准一致。
