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Comparative Study of the Clinical Pathology, Immunophenotype, Epstein–Barr Virus Infection Status, and Gene Rearrangements in Adult and Child Patients With Hydroa Vacciniforme–Like Lymphoproliferative Disorder

Wen, Peng-Fei, MD*; Zhang, Min, MD; Wang, Ting-Ting, MD*; Liu, Hong-Jie, MD*; Zhang, Wen-Yan, MD; Liu, Wei-Ping, PhD; Wang, Lin, PhD*

The American Journal of Dermatopathology: January 2019 - Volume 41 - Issue 1 - p 7–15
doi: 10.1097/DAD.0000000000001229
Original Study
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Background: Hydroa vacciniforme–like lymphoproliferative disorder (HVLPD) is a rare Epstein–Barr virus (EBV)-associated lymphoma that mainly affects children.

Objectives: To examine the similarities and differences in the clinical pathological features, EBV infection status, and gene rearrangements in adults and children patients with HVLPD.

Methods: We compared the clinical manifestations, histopathology, immunophenotypical features, EBV infection status, and T-cell receptor gene rearrangements in the adult and children HVLPD groups.

Results: Clinical manifestations differed between children and adults groups. The children were characterized by blisters and severe facial swelling, whereas the adults were characterized by mild facial swelling and papules. Mosquito bite was significantly related to morbidity in the children group. Histologically, the number of mast cells in the adult group was greater than in the children group (P < 0.05). There were no significant differences in EBV infection status or TCR-γ gene rearrangements between 2 groups.

Conclusions: There were differences in clinical pathology and prognosis between the 2 groups. A higher mast cell count and T-cell phenotype might be associated with a poor prognosis.

*Department of Dermatology, West China Hospital, Sichuan University, Chengdu, Sichuan, China;

Department of Dermatology, The Second People's Hospital of Chengdu, Cheng du, China; and

Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Correspondence: Lin Wang, PhD, Department of Dermatology, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Chengdu, China (e-mail: lkzwl@126.com).

Supported in part by a subject entitled “Study on the correlation between the expression of TOX gene and progression of hydroa vacciniforme–like lymphoproliferative disorder” from the Scientific and Technological Office of Sichuan Province.

P.-F. Wen and M. Zhang have contributed equally to this work.

The authors declare no conflicts of interest.

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