title: T-lymphoblastic lymphoma arising in the small intestine.
abstract: The authors report the clinical, pathological and immunological features of a case of T-lymphoblastic lymphoma presenting with protein-losing enteropathy. There was extensive multifocal involvement of the duodenum, jejunum and ileum. The mediastinum was not enlarged; the peripheral blood picture and bilateral bone marrow trephine biopsies were unremarkable. The tumor cells were positive for terminal deoxynucleotide transferase, CD3, CD2, CD7 and CD10; they were negative for CD1, CD5, CD4, CD8 and HLA-DR. The immunophenotype was that of an immature thymic T-cell. Monocytic and B-cell markers were negative. Despite initial dose reduction in chemotherapy, the patient still developed massive intestinal hemorrhage and succumbed 2 wks after treatment. Postmortem examination confirmed absence of thymic involvement. The overall picture strongly suggests a primary intestinal origin of this T-lymphoblastic lymphoma which contradicts the conventional wisdom that T-lymphoblastic lymphoma arises in the thymus from primitive cortical lymphocytes before rapidly disseminating.
authors.affiliation: Department of Medicine, University of Hong Kong.
authors.forename: E K
authors.initials: EK
authors.lastname: Chiu
authors.forename: S L
authors.initials: SL
authors.lastname: Loke
authors.forename: A C
authors.initials: AC
authors.lastname: Chan
authors.forename: R H
authors.initials: RH
authors.lastname: Liang
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D019011:Antigens, CD7; D000943:Antigens, Differentiation / Q000032:analysis; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis; D000951:Antigens, Neoplasm / Q000032:analysis; D018801:CD2 Antigens; D017252:CD3 Complex; D004379:Duodenal Neoplasms / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007078:Ileal Neoplasms / Q000276:immunology / Q000473:pathology*; D016130:Immunophenotyping; D007580:Jejunal Neoplasms / Q000276:immunology / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000473:pathology*; D008297:Male; D015260:Neprilysin; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000276:immunology / Q000473:pathology*; D011948:Receptors, Antigen, T-Cell / Q000032:analysis; D011971:Receptors, Immunologic / Q000032:analysis
chemical_list: D015703:Antigens, CD; D019011:Antigens, CD7; D000943:Antigens, Differentiation; D000945:Antigens, Differentiation, T-Lymphocyte; D000951:Antigens, Neoplasm; D018801:CD2 Antigens; D017252:CD3 Complex; D011948:Receptors, Antigen, T-Cell; D011971:Receptors, Immunologic; D015260:Neprilysin