Rapidly progressive adult-onset hydroa vacciniforme-like lymphoproliferative disorder associated with Epstein-Barr virus: a case report.
- 1Department of Medicine, University of Maryland Medical Center, Baltimore, MD, USA.
- 2University of Maryland School of Medicine and the Marlene & Stewart Greenebaum Comprehensive Cancer Center, Baltimore, MD, USA.
Abstract
Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein-Barr virus (EBV)-associated T- or natural killer (NK)-cell lymphoproliferative disease that classically affects children. Adult-onset disease is uncommon and may follow an aggressive systemic course. We report a case of systemic adult-onset HVLPD in a 63-year-old woman who initially presented symptoms concerning for an autoimmune process. Initial biopsies demonstrated thrombotic vasculopathy and granulomatous dermatitis without malignant features. Despite transient improvement with immunosuppressive therapy, she later developed rapidly progressive disease and repeat biopsy confirmed and EBV-associated clonal T-cell lymphoproliferative process consistent with systemic HVLPD. Staging studies demonstrated widespread nodal and extranodal disease. The patient received CHOEP chemotherapy followed by nivolumab after early progression; however, the disease remained refractory, and she ultimately elected for hospice care. This case highlights the diagnostic difficulty of adult-onset HVLPD when early manifestations mimic inflammatory or vasculopathic disease.
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BibTeX
@article{deng2026rapidly,
title = {{Rapidly progressive adult-onset hydroa vacciniforme-like lymphoproliferative disorder associated with Epstein-Barr virus: a case report.}},
author = {Joseph Deng and Gideon Wolf and Parth Contractor and Jasmine Pringle and Graeme Fenton and Shella Saint Fleur-Lominy},
journal = {Leukemia research reports},
year = {2026},
month = oct,
doi = {10.1016/j.lrr.2026.100620},
url = {https://europepmc.org/article/MED/42959317},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Rapidly progressive adult-onset hydroa vacciniforme-like lymphoproliferative disorder associated with Epstein-Barr virus: a case report.
AU - Joseph Deng
AU - Gideon Wolf
AU - Parth Contractor
AU - Jasmine Pringle
AU - Graeme Fenton
AU - Shella Saint Fleur-Lominy
PY - 2026
DA - 2026/10/21
JO - Leukemia research reports
DO - 10.1016/j.lrr.2026.100620
UR - https://europepmc.org/article/MED/42959317
AB - Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein-Barr virus (EBV)-associated T- or natural killer (NK)-cell lymphoproliferative disease that classically affects children. Adult-onset disease is uncommon and may follow an aggressive systemic course. We report a case of systemic adult-onset HVLPD in a 63-year-old woman who initially presented symptoms concerning for an autoimmune process. Initial biopsies demonstrated thrombotic vasculopathy and granulomatous dermatitis without malignant features. Despite transient improvement with immunosuppressive therapy, she later developed rapidly progressive disease and repeat biopsy confirmed and EBV-associated clonal T-cell lymphoproliferative process consistent with systemic HVLPD. Staging studies demonstrated widespread nodal and extranodal disease. The patient received CHOEP chemotherapy followed by nivolumab after early progression; however, the disease remained refractory, and she ultimately elected for hospice care. This case highlights the diagnostic difficulty of adult-onset HVLPD when early manifestations mimic inflammatory or vasculopathic disease.
SN - 2213-0489
ER -