Infantile muscle phosphorylase-b-kinase deficiency. A case report |
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Authors: | G Sahin T Güng?r W Rettwitz-Volk W Schlote YS Shin T Podskarbi AC Sewell |
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Affiliation: | Department of Pediatrics and Clinical Immunology, Mie University School of Medicine, Tsu, Japan. |
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Abstract: | A 13-year-old male developed thymic non-Hodgkin's lymphoma. Microscopically, the tumor was composed of large cells, resembling centroblasts. Immunohistochemically, the tumor demonstrated leukocyte common antigen+, L26 (B-cell)+, UCHL1 (T-cell)-, suggesting the B-cell phenotype. In contrast to the terminally differentiated phenotype (CD10-, surface immunoglobulin-) observed in adult cases, flow cytometric analysis showed that they were relatively immature: CD10+, CD19+, HLA-DR-, IgM+/-, kappa+. He was successfully treated with intensive chemotherapy. Since childhood thymic lymphomas are exclusively small non-cleaved cell lymphoma with T-cell phenotype, this case represents a unique entity in children. |
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