The Clinical Analysis Of 15 Children With Hydroa Vacciniforme-like Lymphoproliferative Disorder

Objective:This article reviews the clinical characteristics,treatment and prognosis of Hydroa vacciniforme–like lymphoproliferative disorder in our hospital,hoping to provide relevant clinical evidence.Methods:This retrospective analysis was carried out on the clinical data of all hospitalized patients in our hospital with HVLPD from October2014 to December 2018,consisting of age,sex,clinical characteristics,laboratory and pathological examination,Therapy,outcome and follow-up.Results:1.In this study,Seven(46.67%)were male,eight(53.33%)were female,the ratio was 7:8.The mean age at disease onset for the children in our group was(6.57±4.02)years(range2 to13 years).2.Rural:City = 11: 4.9 children had a history of allergies to mosquito bites,and 12 children developed lesion in summer.3.The clinical features were facial edema,the sun-exposed parts of the face and limbs repeatedly suffered from papules,blisters,ulcers,crust,which healed with atrophic scars.These lesions also occurred in non-exposed areas such as the trunk.Thesepatients usually had systemic symptoms including fever,hepatosplenomegaly and lymphadenopathy.Some patients even suffered from liver and kidney failure and hemophagocytic syndrome(HPS).4.Epstein-Barrvirus-IgG antibody(EBV-IgG)were all positive,Epstein-Barrvirus-IgG antibody(EBV-IgM)were all negative.5.8 patients had abnormal liver function,7 had high LDH,9 had high blood IgE,and 2had bone marrow puncture showing stimulation with phagocytosis.5.Pathological manifestations: epidermal hyperkeratosis,necrosis,spongy edema,blister under the epidermis,focal and dense atypical lymphocytic infiltration of dermal and subcutaneous tissue with perivascular and periadnexal distribution,with varying degrees of atypia.13 cases CD4,CD8(+),7 cases CD56(+),4 cases CD4,CD8,CD56(+),12 cases CD3(+),2cases CD30(+),9 cases TIA-1(+),8 cases granzyme B(+),11 cases of Ki67 proliferation index varied from 3% to 50%,EBER: all positive.3cases of TCR gene rearrangement were negative.6.13 patients were treated with glucocorticoid,13 patients were treated with intramuscular injection of interferon,10 patients were treated with acyclovir,12 patients were treated with gamma globulin,10 patients had recurrence,2 patients had temporary remission,and 3 patients died.Mortality rat(male :female=1:2).Conclusions: 1.HVLPD is closely related to chronic active EBV infection.Most of the children are hypersensitive to mosquito bites in summer.2.Indicators for predicting disease progression include: systemicsymptoms such as liver damage;thrombocytopenia;high LDH;high Ki67 proliferation index;T cell phenotype;high EBV DNA levels and EBV antibody titers in peripheral blood or high EBER positive rate in the biopsy.3.Hypersensitivity to mosquito bites,high IgE titer and relatively indolent clinical course are the main features of patients expressing NK cell phenotype.Patients with T cell phenotype are more likely to progress to systemic disease with poor prognosis.4.Conservative therapies such as:glucocorticoids,gamma globulin,interferon can effectively control symptoms,CHOP chemotherapy can induce temporary remission,but most cases show higher mortality than,so the treatment is not recommended for active chemotherapy.5.Most of the patients from countryside are in high-altitude and high-ultraviolet-irradiated regions,indicating that environmental factors are closely related to HVLPD,so patients with HVLPD should improve their living environment and should be protected from mosquitos and sunlight.