{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T11:32:20Z","timestamp":1792668740902,"version":"4.5.0"},"reference-count":31,"publisher":"American Society of Hematology","issue":"26","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2019,6,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Patients with classic hydroa vacciniforme\u2013like lymphoproliferative disorder (HVLPD) typically have high levels of Epstein-Barr virus (EBV) DNA in T cells and\/or natural killer (NK) cells in blood and skin lesions induced by sun exposure that are infiltrated with EBV-infected lymphocytes. HVLPD is very rare in the United States and Europe but more common in Asia and South America. The disease can progress to a systemic form that may result in fatal lymphoma. We report our 11-year experience with 16 HVLPD patients from the United States and England and found that whites were less likely to develop systemic EBV disease (1\/10) than nonwhites (5\/6). All (10\/10) of the white patients were generally in good health at last follow-up, while two-thirds (4\/6) of the nonwhite patients required hematopoietic stem cell transplantation. Nonwhite patients had later age of onset of HVLPD than white patients (median age, 8 vs 5 years) and higher levels of EBV DNA (median, 1\u2009515\u2009000 vs 250\u2009000 copies\/ml) and more often had low numbers of NK cells (83% vs 50% of patients) and T-cell clones in the blood (83% vs 30% of patients). RNA-sequencing analysis of an HVLPD skin lesion in a white patient compared with his normal skin showed increased expression of interferon-\u03b3 and chemokines that attract T cells and NK cells. Thus, white patients with HVLPD were less likely to have systemic disease with EBV and had a much better prognosis than nonwhite patients. This trial was registered at www.clinicaltrials.gov as #NCT00369421 and #NCT00032513.<\/jats:p>","DOI":"10.1182\/blood.2018893750","type":"journal-article","created":{"date-parts":[[2019,5,7]],"date-time":"2019-05-07T20:25:18Z","timestamp":1557260718000},"page":"2753-2764","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":69,"title":["Hydroa vacciniforme\u2013like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in whites"],"prefix":"10.1182","volume":"133","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0238-7176","authenticated-orcid":false,"given":"Jeffrey I.","family":"Cohen","sequence":"first","affiliation":[{"name":"Laboratory of Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, 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P.","family":"Sarkany","sequence":"additional","affiliation":[{"name":"Department of Photodermatology, St John\u2019s Institute of Dermatology, Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London, United Kingdom;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lori L.","family":"Bonnycastle","sequence":"additional","affiliation":[{"name":"Medical Genomics and Metabolic Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter S.","family":"Chines","sequence":"additional","affiliation":[{"name":"Medical Genomics and Metabolic Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy J.","family":"Swift","sequence":"additional","affiliation":[{"name":"Medical Genomics and Metabolic Genetics Branch, National Human Genome Research 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