AI Article Synopsis

  • * Analysis included 393 patients, revealing that 56% of severe cases were among Asians and showing significant differences in symptoms and outcomes based on race.
  • * Findings indicate that facial edema is a key risk factor for disease progression and survival, with a high mortality rate (39.7%) in severe cases, especially among Latin Americans.

Article 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.

Conclusion: HVLPD is a heterogeneous entity with variable clinicopathological features associated with genetic predispositions.

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http://dx.doi.org/10.1159/000530607DOI Listing

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Article Synopsis
  • * Analysis included 393 patients, revealing that 56% of severe cases were among Asians and showing significant differences in symptoms and outcomes based on race.
  • * Findings indicate that facial edema is a key risk factor for disease progression and survival, with a high mortality rate (39.7%) in severe cases, especially among Latin Americans.
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