Ongoing remission after intensive ALL-type chemotherapy in pediatric intestinal T-cell lymphoma.
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.
Friedrich
Carsten
C
Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Schrum
Johanna
J
Chott
Andreas
A
Janka-Schaub
Gritta
G
Kabisch
Hartmut
H
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D003630:Daunorubicin / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D006801:Humans; D007414:Intestinal Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology*; D008297:Male; D015122:Mercaptopurine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D008775:Methylprednisolone / Q000008:administration & dosage; D009367:Neoplasm Staging; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics; D012074:Remission Induction; D013866:Thioguanine / Q000008:administration & dosage; D014750:Vincristine / Q000008:administration & dosage
D016692:Receptors, Antigen, T-Cell, gamma-delta; D003561:Cytarabine; D014750:Vincristine; D005047:Etoposide; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide; D015122:Mercaptopurine; D013866:Thioguanine; D008775:Methylprednisolone; D008727:Methotrexate; D003630:Daunorubicin