Case Report and Review of Literature of Child Subcutaneous Panniculitis-Like T-Cell Lymphoma / 实用儿科临床杂志

ABSTRACT

Objective To analyze the clinical and histopathologic characteristics in children with subcutaneous panniculitis-like T-cell lymphoma(SPTCL),and explore the pathological diagnosis and differential diagnosis,in order to boost paediatricians to better understanding the disease.Methods One case was diagnosed SPTCL with over 2 years protracted course of fever and multiple skin lesions.The evolvement of clinical presentation,the misdiagnosed experience,the histopathological features,the immunohistochemical results and T cell receptor(TCR)gene cloning rearrangement were observed.The related literatures published were reviewed.Results Skin biopsy showed that the histopathologic findings were limited within subcutaneous fatty tissue,the atypical lymphocytes characteristically rimmed individual fat cells in a lace-like pattern.Immunophenotypic studies showed CD45,CD45RO,CD3,CD5,T cell intracellular antigen-1(TIA-1)and perforin usually expressed possitive,while CD10,CD20,CD56,CD68,epithelial membrane antigen(EMA)and cytokeratin(CK)were negative,implying tumor cells derived from T-cell.The results of TCR gene rearrangement as followingIgH FR2(+),FR3A(-);TCR ? JVI(-),JVII(+);TCR? JD1(-),JD2(-).Although the protocol of children's T-cell Non-Hodgkin lymphoma was administrated,the treatment outcome was poor.Conclusion SPTCL is a special primary cutaneous lymphoma with poor prognosis,skin biopsy of suffered lesion is an important method for the diagnosis of SPTCL children with unclear recurrent fever and multiple skin lesions.