■水痘病毒(HV)淋巴增殖性疾病是一种罕见的NK/T细胞淋巴瘤,主要影响儿童,临床上与HV相似,主要在拉丁美洲和一些亚洲国家报道。总的来说,成熟的T细胞和NK细胞肿瘤现在根据不同的概念分为9个家族:细胞起源/分化状态,临床情景,疾病定位,和细胞形态学。根据世界卫生组织成熟淋巴肿瘤分类的第五版,HV淋巴增生性疾病被列为EbsteinBarr病毒阳性T细胞和NK细胞淋巴增生和儿童淋巴瘤。我们报道了一名22岁白人妇女的特殊情况,土生土长的西班牙,第一次出现在2016年,当时她开始患有复发性面部水肿。四年后,该疾病进展为淋巴结扩散和致命的结果。这里,我们描述了淋巴瘤在其演变过程中的临床和组织学表现.像这样的病例很难分类,这对临床医生和病理学家构成了真正的挑战。所以,必须意识到这种疾病的罕见表现,以便能够识别临床和组织学图片,以做出正确的诊断并建立早期治疗。
UNASSIGNED: Hydroa vacciniforme (HV) lymphoproliferative disorder is a rare NK/T-cell lymphoma mainly affecting children and with a clinical resemblance to HV, which is mostly reported in Latin American and some Asian countries. Overall, the mature T cell and NK-cell neoplasms are now grouped into 9 families based on diverse concepts: cell of origin/differentiation state, clinical scenario, disease localization, and cytomorphology. HV lymphoproliferative disorder is listed within the group of Ebstein Barr Virus-positive T-cell and NK-cell lymphoid proliferations and lymphomas of childhood according to the fifth edition of the World Health Organization Classification of mature lymphoid neoplasms. We report the extraordinary
case of a 22-year-old white woman, native of Spain, first presented in 2016 when she started suffering from recurrent facial edema. Four years later, the disease progressed with lymph node spreading and a fatal outcome. Here, we describe the clinical and histological presentation of the lymphoma throughout its evolution. Cases like this can be difficult to classify posing a real challenge to clinicians and pathologists. So, it is vital to be aware of the rare presentation of this disease to be able to identify the clinical and histological picture to make a correct diagnosis and establish an early treatment.