Clinical and pathological analysis on hydroa vacciniforme-like lymphoproliferative disorder in children / 临床与实验病理学杂志

ABSTRACT

Purpose To discuss the clinical,histopathological characteristics,diagnosis,differential diagnosis and prognosis of hydroa vacciniforme-like lymphoproliferative disorder in children.Methods 6 cases of hydroa vacciniforme-like lymphoproliferative disorder were analyzed by molecular,histopathological and immunohistochemical testing.Clinical and follow-up information was obtained.The published relevant literatures were reviewed.Results 4 cases were boys,2 case were girls.All the patients presented with erythema and blisters with fever for 1 month to 4 years.Histopathologic examination showed an mild or moderate atypical lymphocytic infiltrate with angiotropism and angiocentricity,and scattered or dense lymphoid infiltration throughout the dermis and subcutaneous tissue.Blisters or necrosis could be seen in the epidermis.The atypical lymphocytes were positive for CD2,CD3,CDS,CD7,CD43,TIA-1,CD4 or CD8,and negative for CD20,Pax-5.Only one case showed positive stain for CD56.The average positive rate of Ki67 in tumor cells was 42.3％.Tumor cells positive for EBV encoded RNA (EBER) were detected in cutaneous infiltrates in 5 cases.Gene rearrangement of TCR was detected in 2 cases.5 patients were available for follow-up examination and 1 patient was dead.Conclusion Hydroa vacciniforme-like lymphoproliferative disorder is a rare disease mainly occuring in children.Chronic active EBV infection has been associated with this disease.It may be a spectrum in terms of its clinical course,and may be benign,borderline and malignant.Pathological diagnosis should be closely combined with clinical data.