| 000 | 02400cam a2200253 4500500 | ||
|---|---|---|---|
| 005 | 20250121155318.0 | ||
| 041 | _afre | ||
| 042 | _adc | ||
| 100 | 1 | 0 |
_aZhang, Yimeng _eauthor |
| 700 | 1 | 0 |
_a Liu, Yuanxiang _eauthor |
| 700 | 1 | 0 |
_a Xu, Jiaosheng _eauthor |
| 700 | 1 | 0 |
_a Liang, Yuan _eauthor |
| 700 | 1 | 0 |
_a Xu, Zigang _eauthor |
| 245 | 0 | 0 | _aHydroa vacciniforme lymphoproliferative disorder: a retrospective study of 42 paediatric cases |
| 260 | _c2024. | ||
| 500 | _a26 | ||
| 520 | _aBackgroundHydroa vacciniforme lymphoproliferative disorder (HVLPD) is a rare disease related to Epstein-Barr virus (EBV), mainly in children, and is an EBV-associated cutaneous T and natural killer (NK) cell lymphoproliferative disorder. The disorder in some patients may progress to EBV-associated systemic T or NK-cell lymphoma.ObjectivesTo summarize the characteristics of HVLPD in Chinese paediatric patients and to examine the risk factors indicating poor prognosis.Materials & MethodsWe performed a retrospective analysis of patients with HVLPD from the Department of Dermatology, Beijing Children’s Hospital. Based on diagnosis, medical history, examination results, and immunophenotype, we analysed HVLPD in 42 paediatric cases in order to examine the clinical features, prognoses, and risk factors.ResultsForty-two paediatric patients were enrolled, with a median onset age of five years. All patients presented with papulovesicular lesions, and 32 systemic HVLPD (sHVLPD) patients had systemic symptoms, including fever, lymphadenopathy, hepatomegaly, splenomegaly, and liver dysfunction. Of the sHVLPD cases, 13 also had severe mosquito bite allergy (SMBA). Twenty-five cases were T-type, and nine were CD56+-dominant type. Follow-up data showed that 12 patients had complete remission, and three patients died.ConclusionSMBA is a risk factor for disease progression in patients with HVLPD, and the pathological CD56+-dominant phenotype is associated with poor prognosis. | ||
| 690 | _ahydroa vacciniforme lymphoproliferative disorder (HVLPD) | ||
| 690 | _asevere mosquito bite allergy (SMBA) | ||
| 690 | _ahydroa vacciniforme (HV) | ||
| 690 | _aEpstein-Barr virus (EBV) | ||
| 786 | 0 | _nEuropean Journal of Dermatology | 34 | 2 | 2024-03-01 | p. 144-149 | 1167-1122 | |
| 856 | 4 | 1 | _uhttps://shs.cairn.info/revue-european-journal-of-dermatology-2024-2-page-144?lang=en&redirect-ssocas=7080 |
| 999 |
_c604432 _d604432 |
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