A Case of CD57-positive Mediastinal T-cell Non-Hodgkin's Lymphoma. Its Malignant Features and Review of the Literature.:Its Malignant Features and Review of the Literature

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A 7-year-old girl was admitted due to cough, fever, chest pain and facial edema. Her left lung was collapsed by the remarkable pleural effusion. The anterior mediastinal tumor shadow became clear radiographically after the drainage of the pleural fluid, which was found to contain numerous lymphoblasts with positivity of monoclonal antibodies such as CD 1, 2, 3, 4, 5, 7, 8, 38 and 57. With the diagnosis of the mediastinal T-cell non-Hodgkin's lymphoma, she was treated with chemotherapy resulting in no remarkable shrinkage of her anterior mediastinal tumor and she died of infections. With a marked contrast, five patients with CD57-T-cell malignancy treated during this period had good responses by chemotherapy similar to the regimen prepared for the CD57+ patient. Natural killer cell-associated surface antigen (NK-At) -positive non-Hodgkin's lymphoma should be distinguished as a biologic subtype from the NK-At-negative T-cell non-Hodgkin's lymphoma because of its poor response to chemotherapy. Especially in this subtype, CD57+ cases are more frequently seen in young females with the anterior mediastinal tumor. No similar case has yet been reported in Japan. A special strategy should be considered for more effective treatment on this tumor.
特定非営利活動法人　日本小児血液・がん学会の論文