A study of subcutaneous panniculitis-like T-cell lymphoma in children

Objective To investigate the clinicopathologic features and immunohistochemical profiles of,as well as gene rearrangement and Epstein-Barr virus(EBV)infection in subcutaneous panniculitis-like T-cell lymphoma(SPTL)in children.Methods Five cases of SPTL in children were analyzed clinico- pathologically.Immunohistochemical staining forβF1,CD2,CD3,CD45RO,CD4,CD8,CD20,CD30, CD56,TIA-1 and granzyme B were performed.PCR was used to assess the rearrangement of TCRγand IgH genes.The EBV infection was detected by in situ hybridization with EBER1/2.Results There were 4 male patients and 1 female patient,ranging in age from 9 to 14 years.The main clinical manifestations were asymptomatic nodules,plaques and masses.Histologically,a panniculitis-like infiltration was observed in the subcutaneous fat;the infiltrating cells varied in size and showed obvious atypia;there were beanbag cells, epithelioid granulomas and lamellar necrosis in the tumor tissues.These tumor cells expressedβF1,CD2, CD3,CD8,CD45RO,TIA-1 and granzyme B,but did not express CD4 or CD20.Monoclonal TCRγgene rearrangement was found in 4 of the 5 cases,while IgH gene rearrangement in none of them.EBV infection was found in 2 cases.Of the three patients available for follow up evaluation,one,who was positive for EBR1/2 died of SPTL.Conclusions In SPTL in children,face is apt to be involved,systemic findings are commonly seen,and hemophagocytic syndrome and death occur at a rather high rate.The prognosis may be poor in SPTL in children associated with EBV infection.