Hydroa vacciniforme-like lymphoproliferative disorder: an EBV disease with a low risk of systemic illness in whites

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- 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.

Source: Blood - June 26, 2019 Category: Hematology Authors: Cohen, J. I., Manoli, I., Dowdell, K., Krogmann, T. A., Tamura, D., Radecki, P., Bu, W., Turk, S.-P., Liepshutz, K., Hornung, R. L., Fassihi, H., Sarkany, R. P., Bonnycastle, L. L., Chines, P. S., Swift, A. J., Myers, T. G., Levoska, M. A., DiGiovanna, J. Tags: Lymphoid Neoplasia, Clinical Trials and Observations Source Type: research