Ongoing remission after intensive All-type chemotherapy in pediatric intestinal T-cell lymphoma

• Published in: Pediatric blood & cancer 2010-04, Vol.54 (4), p.610-612
• Main Authors: Friedrich, Carsten, Schrum, Johanna, Chott, Andreas, Janka-Schaub, Gritta, Kabisch, Hartmut
• Format: Article
• Language: English
• Subjects: Acute lymphatic leukemia; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Blood; Cancer; CD3 antigen; CD4 antigen; CD8 antigen; Celiac disease; Chemotherapy; Child; Cyclophosphamide - administration & dosage; Cytarabine - administration & dosage; Daunorubicin - administration & dosage; Dexamethasone - administration & dosage; Doxorubicin - administration & dosage; enteropathy-associated T-cell lymphoma; Epstein-Barr virus; Etoposide - administration & dosage; Humans; Infection; Intestinal Neoplasms - drug therapy; Intestinal Neoplasms - genetics; Intestinal Neoplasms - pathology; Intestine; Lymphocytes; Lymphoma, T-Cell - drug therapy; Lymphoma, T-Cell - genetics; Lymphoma, T-Cell - pathology; Male; Mercaptopurine - administration & dosage; Methotrexate - administration & dosage; Methylprednisolone - administration & dosage; Neoplasm Staging; pediatric; Pediatrics; Receptors, Antigen, T-Cell, gamma-delta - genetics; Remission; Remission Induction; Risk factors; T-cell lymphoma; T-cell receptor; Thioguanine - administration & dosage; Tumor cells; Vincristine - administration & dosage
• ISSN: 1545-5009; 1545-5017; 1545-5017
• DOI: 10.1002/pbc.22330

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 βF1+, 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 γ‐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. Pediatr Blood Cancer 2010;54:610–612. © 2009 Wiley‐Liss, Inc.