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Primary Cutaneous Extranodal NK/T-cell Lymphoma, Extranasal Type in Child: A Case Report and Literature Review
Author(s): 
Pages: 20-24
Year: Issue:  1
Journal: Chinese Journal of Dermatovenerology of Integrated Traditional and Western Medicine

Keyword:  NK/T-Cell LymphomaExtranasal typeEpstein-Barr virus;
Abstract: Objective In order to report a case of primary cutaneous extranodal NK/T-cell lymphoma,extranasal type,and the literatures are reviewed.Methods The clinical manifestation,laboratory examinations,tissue pathology and immunohistochemical staining of a case of primary cutaneous extranodal NK/T-cell lymphoma,extranasal type in a child were investigated and analyzed.Results A 11-year-old boy presented with subcutaneous nodules and plaques for a month.Severve hepatomegaly,splenomegaly,and lymphadenectasis were found in physical examination.Blood routine test indicated significantly decreased number of WBC,PLT,and notably increased level of AST,LDH was recorded.Impaired blood coagulation,high replication of EBV genes was detected.The histopathology of skin lesion exhibited that subcutaneous tissue was infiltrated by diffuse or dense lymphoid cells with atypia which were also detected in left axillary node.Immunohistochemical staining showed that CD3,CD56,GramB and TIA-1 were positive,Ki-67 was about 30% to 40% positive.EBV-encoded RNA (EBER) examinated by in-situ hybridization was positive both in skin lesion and left axillary lymph node.Conclusion Given the clinical manifestation is unspecific,the histopathology examination,the definitive markers of immunohistochemical staining and characteristic changes of EBER is important to establish the diagnosis.Primary cutaneous extranodal NK/T cell lymphoma,extranasal type,with a high-grade malignancy,intends to poor prognosis.
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