Abstract：
BACKGROUND: Limited clinicopathological and prognostic data are available on hydroa vacciniforme (HV)-like lymphoproliferative diseases (HVLPD).
METHODS: This systematic review searched HVLPD reports in Medline via PubMed, Embase, Cochrane, and CINAHL databases in October 2020.
RESULTS: A total of 393 patients (65 classic HV, 328 severe HV/HV-like T-cell lymphoma [HVLL]) were analyzed. Among severe HV/HVLL cases, 56.0% were Asians, whereas 3.1% were Caucasians. Facial edema, hypersensitivity to mosquito bites, the onset of skin lesion, and percentage of severe HV/HVLL differed significantly by race. Progression to systemic lymphoma was confirmed in 9.4% of HVLPD patients. Death occurred in 39.7% patients with severe HV/HVLL. Facial edema was the only risk factor associated with progression and overall survival. Mortality risk was higher in Latin Americans than in Asians and Caucasians. CD4/CD8 double-negativity was significantly associated with the worst prognosis and increased mortality.
CONCLUSIONS: HVLPD is a heterogeneous entity with variable clinicopathological features associated with genetic predispositions.
METHODS: This systematic review searched HVLPD reports in Medline via PubMed, Embase, Cochrane, and CINAHL databases in October 2020.
RESULTS: A total of 393 patients (65 classic HV, 328 severe HV/HV-like T-cell lymphoma [HVLL]) were analyzed. Among severe HV/HVLL cases, 56.0% were Asians, whereas 3.1% were Caucasians. Facial edema, hypersensitivity to mosquito bites, the onset of skin lesion, and percentage of severe HV/HVLL differed significantly by race. Progression to systemic lymphoma was confirmed in 9.4% of HVLPD patients. Death occurred in 39.7% patients with severe HV/HVLL. Facial edema was the only risk factor associated with progression and overall survival. Mortality risk was higher in Latin Americans than in Asians and Caucasians. CD4/CD8 double-negativity was significantly associated with the worst prognosis and increased mortality.
CONCLUSIONS: HVLPD is a heterogeneous entity with variable clinicopathological features associated with genetic predispositions.
摘要：
背景：关于生鱼水状（HV）样淋巴组织增生性疾病（HVLPD）的临床预后数据有限。
方法：本系统评价通过PubMed搜索了Medline的HVLPD报告，Embase,科克伦,2020年10月和CINAHL数据库。
结果：共有393例患者（65例经典HV，分析了328例严重的HV/HV样T细胞淋巴瘤[HVLL]）。在严重的HV/HVLL病例中，56.0%是亚洲人，而3.1%是白种人。面部水肿,对蚊虫叮咬过敏,皮肤病变的发生和严重HV/HVLL的百分比因种族而异。在9.4%的HVLPD患者中证实了向全身性淋巴瘤的进展。39.7%的重度HV/HVLL患者死亡。面部水肿是与疾病进展和总生存期相关的唯一危险因素。拉丁美洲人的死亡风险高于亚洲人和高加索人。CD4/CD8双阴性与预后不良和死亡率增加显著相关。
结论：HVLPD是一种异质性实体，具有与遗传易感性相关的可变临床病理特征。
方法：本系统评价通过PubMed搜索了Medline的HVLPD报告，Embase,科克伦,2020年10月和CINAHL数据库。
结果：共有393例患者（65例经典HV，分析了328例严重的HV/HV样T细胞淋巴瘤[HVLL]）。在严重的HV/HVLL病例中，56.0%是亚洲人，而3.1%是白种人。面部水肿,对蚊虫叮咬过敏,皮肤病变的发生和严重HV/HVLL的百分比因种族而异。在9.4%的HVLPD患者中证实了向全身性淋巴瘤的进展。39.7%的重度HV/HVLL患者死亡。面部水肿是与疾病进展和总生存期相关的唯一危险因素。拉丁美洲人的死亡风险高于亚洲人和高加索人。CD4/CD8双阴性与预后不良和死亡率增加显著相关。
结论：HVLPD是一种异质性实体，具有与遗传易感性相关的可变临床病理特征。
