Primary CD20+CD10+CD8+ T-cell lymphoma of the skin with IgH and TCR beta gene rearrangement.
Most cutaneous T-cell lymphomas are derived from mature postthymic T cells of the CD4 subtype. When other less common profiles are encountered, a diagnostic challenge is posed. Accurate categorization is critical because of the specificity of therapeutic regimens, including biologics. A 65-year-old woman was seen in 2001 because of a thigh mass manifesting an unusual phenotype eventually categorized as a mature postthymic CD8+ T-cell lymphoma with CD10 and weak CD20 expression. Molecular studies revealed T-cell receptor and heavy chain immunoglobulin rearrangement. Her cutaneous disease progressed despite several cycles of chemotherapy and radiation therapy. However, a therapeutic trial with denileukin diftitox resulted in a striking response. The importance of this case lies in the novel phenotype and dual T- and B-cell rearrangements. Rather than representing an aberrant phenotype, this tumor may represent the malignant counterpart of a benign population of weakly CD20+ T cells of the CD8 subset.
Magro
Cynthia M
CM
Department of Pathology, The Ohio State University, Columbus, USA.
Seilstad
Kay H
KH
Porcu
Pierluigi
P
Morrison
Carl D
CD
D000368:Aged; D015703:Antigens, CD / Q000378:metabolism*; D018951:Antigens, CD20 / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016827:CD8 Antigens / Q000378:metabolism; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain / Q000235:genetics*; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor / Q000235:genetics*; D019674:Genes, T-Cell Receptor beta / Q000235:genetics; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics*; D016130:Immunophenotyping / Q000379:methods; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy / Q000235:genetics* / Q000378:metabolism; D009364:Neoplasm Recurrence, Local; D015260:Neprilysin / Q000378:metabolism; D011239:Prednisolone / Q000008:administration & dosage; D012878:Skin Neoplasms / Q000188:drug therapy / Q000235:genetics* / Q000378:metabolism; D016176:T-Lymphocyte Subsets / Q000276:immunology / Q000473:pathology; D014750:Vincristine / Q000008:administration & dosage
D015703:Antigens, CD; D018951:Antigens, CD20; D016827:CD8 Antigens; D007143:Immunoglobulin Heavy Chains; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone; D015260:Neprilysin