Abstract:
:Patients with classic hydroa vacciniforme-like 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, 1 515 000 vs 250 000 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-γ 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.
journal_name
Bloodjournal_title
Bloodauthors
Cohen JI,Manoli I,Dowdell K,Krogmann TA,Tamura D,Radecki P,Bu W,Turk SP,Liepshutz K,Hornung RL,Fassihi H,Sarkany RP,Bonnycastle LL,Chines PS,Swift AJ,Myers TG,Levoska MA,DiGiovanna JJ,Collins FS,Kraemer KH,Pittaludoi
10.1182/blood.2018893750subject
Has Abstractpub_date
2019-06-27 00:00:00pages
2753-2764issue
26eissn
0006-4971issn
1528-0020pii
blood.2018893750journal_volume
133pub_type
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