Abstract
A rare case of primary intestinal T-cell lymphoma (ITL) of an 8-year-old boy is reported. Medium- to large-sized tumor cells were betaF1+, CD3+, CD8+. TIA-1+, but CD4-, CD5-, CD30-, CD56-, CD20-, CD79a-, TdT-, consistent with an intraepithelial lymphocyte (IEL) origin. They showed monoclonal rearrangement of the T-cell receptor gamma-chain and no evidence of EBV infection. No clinical, histologic, laboratory, or genetic evidence of celiac disease was detected. In adults, ITL is often associated with enteropathy and has a very poor outcome. Our patient remains in first remission 30 months after finishing the acute lymphoblastic leukemia protocol COALL-07-03 high risk standard.
MeSH terms
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
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Child
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Cyclophosphamide / administration & dosage
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Cytarabine / administration & dosage
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Daunorubicin / administration & dosage
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Dexamethasone / administration & dosage
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Doxorubicin / administration & dosage
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Etoposide / administration & dosage
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Humans
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Intestinal Neoplasms / drug therapy*
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Intestinal Neoplasms / genetics
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Intestinal Neoplasms / pathology*
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Lymphoma, T-Cell / drug therapy*
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Lymphoma, T-Cell / genetics
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Lymphoma, T-Cell / pathology*
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Male
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Mercaptopurine / administration & dosage
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Methotrexate / administration & dosage
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Methylprednisolone / administration & dosage
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Neoplasm Staging
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Receptors, Antigen, T-Cell, gamma-delta / genetics
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Remission Induction
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Thioguanine / administration & dosage
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Vincristine / administration & dosage
Substances
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Receptors, Antigen, T-Cell, gamma-delta
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Cytarabine
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Vincristine
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Etoposide
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Dexamethasone
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Doxorubicin
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Cyclophosphamide
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Mercaptopurine
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Thioguanine
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Methylprednisolone
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Methotrexate
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Daunorubicin