Supplementary Material for: Clinicopathologic and prognostic study of hydroa vacciniforme-like lymphoproliferative diseases: A systematic review

Background: Limited clinicoprognostic 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. Conclusion: HVLPD is a heterogeneous entity with variable clinicopathologic features associated with genetic predispositions.