The Clinic Analysis Of 13 Cases Of Hydroavacciniforme-like Lymphoproliferative Disorder In Children

Background and PurposeHydroavacciniforme-like lymphoproliferative disorder(HVLPD)is a rare lymphoma with skin lesions associated with Epstein-Barr virus infection,primarily in children.In 2016,the World Health Organization(WHO)named it HVLLLPD.HVLPD occurs mainly in children in Asia and Latin America,and there is no clear gender bias.The appearance of lesions is similar to Hydroa Vacciniforme(HV),but the clinical course is different.Patients may have recurrent skin lesions in both sunny and nonsunny areas with systemic symptoms including fever,lymphadenopathy,and hepatosplenomegaly.Although there have been some cases in children in recent years,the low incidence of HVLLLPD makes it difficult to collect clinical data.Therefore,in this retrospective study,we collected the case data of 13 patients with HVLPD,and analyzed the clinical characteristics,histophology,immunophenotype,EBV infection status and T cell receptor(TCR)gene rearrangement of HVLPD patients to better understand the disease.Materials and MethodsWe respectively analyzed the clinical case data of 13 children which were diagnosed with hydroa vacciniforme-like lymphoproliferative disease in September 2012 to July 2019 in the first affiliated hospital of zhengzhou university.The case data include children age,gender,clinical symptoms,signs,clinical laboratory results,histopathological manifestation,treatment and outcome.Results1.General clinical data:Among the 13 children,7 were male,6 were female(the ratio of male to female is 1.71:1).The age ranged from 2 to 14 years old.Only a child’s age was under 3 years old.6 children were 3-6 years old,4 were 6-12 years old,and 2 were over 12 years old.The median age of treatment was 7 years old.The course of the disease raged from 11 months to 6 years,including a case lower than 1 year,6 cases 1-3 years,4 cases 3-5years and 2 cases more than 5 years.2.Symptoms and signs:13 children were treated for skin lesions with varying degree of severity.7 children had fever and 8 children had lymphonodepathy.5 cases were associated with hepatomegaly and(or)splenomegaly.7 cases were aggravated by mosquito bites,presenting as new herpes with fever.4 children had a history of photoallergy.3.Auxilliary examination:Among the 13 children,EBV quantification increased in 10 cases.LDH slightly increased in 6 cases.6 children had different IgE levels.Liver function transaminase increased slightly in 9 children.The inflammatory index was slightly increased in 12 children.The results of bone marrow puncture in 2 children were negative.4.Immunophenotype and histopathology:The immunohistochemistry,EBER,and TCR of 13 children were positive(+).11 children were T-cell phenotype and 2 children were NK-cell phenotype.The index of Ki-67 increased in varying degrees(10%-100%).All children were CD3 positive,8 children were CD4 and CD8 positive,7 children were CD56 positive,4 children were CD4,CD8 and CD 56 positive,and 6 children express CD30.10 cases express TIA-1 and 11 cases express granular enzyme B.Microscopically,there were varying degrees of dense,patchy,nodular,or diffuse lymphocytic infiltrations througout the dermis.The subcutaneous tissue is dense and dominated by lobules.Epidermal necrosis was observed in 9 children.The infiltration was vascular centrality.The infiltrating lymphocytes are of different sizes,mainly small and medium-sized cells,mixed with some large lymphocytes.The atypia of cell is not obvious.5.Treatment and prognosis:All the 13 children received conservative treatment such as antiviral,glucocorticoid,immunosuppreaant and anti-infection initially,and the early effect was good.The rash subsided and healed.The quantitative determination of EBV DNA in peripheral blood of 2 children was normal after conservative treatment and follow-up.4 cases suffered from repeated recurrence due to mosquito bites,and 7 cases suffered recurrence for unknown reasons.8 cases was relieved again by giving conservative treatment including adjusting doses of glucocorticoid and cyclosporine and strengthening antiviral treatment.Etoposide and methotrexate was used to treated a children due to the poor efficacy of conservative treatment,and the children’s condition was relieved.1 cases received DDGP chemotherapy due to the progression of aggressive NK/T-cell lymphoma,and a reccurrent patient recevied CHOP chemotherapy due to the progression of EBV+T-cell lymphoma.During the median 48-month follow up,2 cases was cured,2 cases died of infection due to chemotherapy,and 9 cases survived with the disease.Conclusion1.Hydroa vacciniforme-like lymphoproliferative disease is a rare clinical EBV-associated cutaneous T-cell lymphoma,which is dominated by T-cell phenotype and rare by NK-cell type.2.The clinical manifestations are recurrent skin lesions similar to hydroa vacciniforme,which may also be involved in the unexposed parts.Some children have systemic symptons such as fever,hepastosplenomegaly and lymph node enlargement,and mosquito bites can lead to recurrent diseases.3.Hydroa vacciniforme-like lymphoproliferative disease has a long course and varied prognosis.Most patients survive with the disease,a few can be cured,a few wiht a long course can progress to malignant lymphoma.