关键词: Chronic active Epstein-Barr virus infection EBV-associated T- or NK-cell lymphoproliferative diseases Facial skin eruption Hydroa vacciniforme lymphoproliferative disorder

Mesh : Male Humans Adult Epstein-Barr Virus Infections Hydroa Vacciniforme / pathology Herpesvirus 4, Human Delayed Diagnosis Exanthema Lymphoproliferative Disorders

来  源:   DOI:10.11406/rinketsu.64.209

Abstract:
Chronic active Epstein-Barr virus (CAEBV) infection is characterized by persistent EBV infection and can lead to fatal conditions such as hemophagocytic syndrome and malignant lymphoma through the clonal expansion of EBV-infected T or natural killer (NK) cells. Hydroa vacciniforme lymphoproliferative disorder (HV) and hypersensitivity to mosquito bites (HMB) have been identified as skin diseases in EBV-associated T- or NK-cell lymphoproliferative diseases. We present the case of a 33-year-old man. The patient had frequent episodes of a facial rash for three years before he visited our hospital, he visited several dermatologists but did not receive a diagnosis of HV. He was referred to the hematology department of our hospital for assessment of atypical lymphocytes in peripheral blood. Based on routine blood and bone marrow test we were unable to diagnose HV. However, when the patient\'s liver function deteriorated six months later, we considered the possibility of HV after reevaluating the skin rash. After performing EBV-related tests, we were able to definitively diagnose CAEBV with HV. It is crucial to be able to connect clinical observations to EBV-related tests when diagnosing CAEBV. Hematologists must be knowledgeable of the EBV-associated skin conditions of HV and HMB.
摘要:
慢性活动性爱泼斯坦-巴尔病毒(CAEBV)感染的特征是持续的EBV感染,并可通过EBV感染的T或自然杀伤(NK)细胞的克隆扩增导致致命的疾病,例如噬血细胞综合征和恶性淋巴瘤。在EBV相关的T-或NK-细胞淋巴增殖性疾病中,已经确定了对蚊虫叮咬的超敏反应(HMB)为皮肤病。我们介绍了一个33岁男子的案例。病人在到我们医院就诊前曾有三年频繁的面部皮疹发作,他访问了几位皮肤科医生,但没有得到HV的诊断。他被转诊到我们医院的血液科,以评估外周血中的非典型淋巴细胞。根据常规血液和骨髓检查,我们无法诊断HV。然而,当病人的肝功能恶化6个月后,我们在重新评估皮疹后考虑了HV的可能性.进行EBV相关测试后,我们能够明确诊断有HV的CAEBV.在诊断CAEBV时,将临床观察与EBV相关的测试联系起来至关重要。血液学家必须了解与EBV相关的HV和HMB的皮肤状况。
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