PMID: 9537480Apr 16, 1998Paper

Abdominal T-cell non-Hodgkin's lymphoma of the gamma/delta type in a patient with selective immunoglobulin A deficiency

The American Journal of Surgical Pathology
M M OttH K Müller-Hermelink

Abstract

A 28-year-old man presented with selective immunoglobulin A deficiency and severe diarrhea responding to a gliadin-free diet. Biopsy samples of the small intestine showed dense T-cell infiltrations in the lamina propria and a slight increase of intraepithelial T-lymphocytes. No clonal rearrangement of the T-cell receptor c-beta chain genes was detectable by Southern blotting. Four years later, at the age of 32, the patient was hospitalized again with liver failure, abdominal lymphadenopathy, pancytopenia, and recurrent bacterial infections. Retrospective polymerase chain reaction analysis of formalin-fixed tissues of the intestinal biopsy samples obtained 4 years earlier showed monoclonal T-cell receptor gamma-chain gene rearrangement. Lymphoid cells of the peripheral blood showed an immunophenotype of CD3-positive gamma/delta T cells with a negativity for CD4 and CD8. A clonally rearranged T-cell receptor delta chain gene and a germline configuration of the c-beta chain genes was found by Southern blotting. Cytogenetics showed an abnormal karyotype with unbalanced translocations t(1;5) and t(9;13). The patient died of extensive lung infiltrations by gamma/delta T cells; autopsy showed a peripheral T-cell lymphoma of the gamma/...Continue Reading

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Citations

Apr 12, 2000·Histopathology·C de Wolf-Peeters, R Achten
Nov 9, 2001·Leukemia & Lymphoma·P KanavarosK Stefanaki
Oct 6, 2016·Frontiers in Immunology·Esmaeil MortazIan M Adcock
Aug 4, 2001·International Journal of Surgical Pathology·R Drut, R M Drut
Dec 1, 2009·Current Protocols in Pharmacology·Joseph R Maxwell, Joanne L Viney
Aug 6, 2016·Journal of Clinical Immunology·Hikmet G TanyildizNurdan Tacyildiz
May 1, 2021·Biology·Alessandra TiriAndrea Pession

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