Hydroa vacciniforme lymphoproliferative disorder: a retrospective study of 42 paediatric cases

• DOI: 10.1684/ejd.2024.4648
• Publication Date: 2024-06-18T05:45:30Z
• Authors: Yimeng Zhang; Yuanxiang Liu; Jiaosheng Xu; Yuan Liang; Zigang Xu
• Keywords: Lymphoproliferative Disorders; Immunophenotyping

Hydroa vacciniforme lymphoproliferative disorder (HVLPD) is a rare disease related to Epstein-Barr virus (EBV), mainly in children, and an EBV-associated cutaneous T natural killer (NK) cell disorder. The some patients may progress systemic or NK-cell lymphoma. To summarize the characteristics of HVLPD Chinese paediatric examine risk factors indicating poor prognosis. We performed retrospective analysis with from Department Dermatology, Beijing Children's Hospital. Based on diagnosis, medical history, examination results, immunophenotype, we analysed 42 cases order clinical features, prognoses, factors. Forty-two were enrolled, median onset age five years. All presented papulovesicular lesions, 32 (sHVLPD) had symptoms, including fever, lymphadenopathy, hepatomegaly, splenomegaly, liver dysfunction. Of sHVLPD cases, 13 also severe mosquito bite allergy (SMBA). Twenty-five T-type, nine CD56+-dominant type. Follow-up data showed that 12 complete remission, three died. SMBA factor for progression HVLPD, pathological phenotype associated