INTESTINAL LYMPHOMA ASSOCIATED WITH MALABSORPTION

doi:10.1016/S0140-6736(78)90004-1

The Lancet

Volume 311, Issue 8055, 14 January 1978, Pages 67-70

https://doi.org/10.1016/S0140-6736(78)90004-1 Get rights and content

Abstract

Of 18 patients with small-intestinal lymphoma associated with villous atrophy and crypt hyperplasia of uninvolved mucosa, most presented with acute obstruction or perforation preceded by abdominal pain and weight-loss. Malabsorption, thought to be adult cœliac disease, preceded the diagnosis of lymphoma in 5 cases. The lymphomas were composed of malignant histiocytes and were classified on morphological grounds as malignant histiocytosis. Of particular interest was the finding by immunohistochemical techniques of all major immunoglobulin classes within malignant cells of single tumours. Characterisation of this group of lymphomas as a specific entity should help in the further understanding of the relationship of malabsorption and lymphoma and in the rationalisation of treatment.

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The IELs frequently spread to other GI organs (e.g., stomach, colon) in RCD II and can also traffic to extra-GI sites (e.g., skin, lungs, CNS) (Malamut et al., 2009, 2013). Epithelial ulcers with acute inflammatory exudate and granulation tissue containing lymphocytes, plasma cells, and macrophages (“ulcerative jejunitis” or “chronic jejuno-ileitis”), can be seen in both RCD I and RCD II, but are more common in RCD II (Ashton-Key et al., 1997; Bagdi et al., 1999; Cellier et al., 2000; Isaacson and Wright, 1978; Jewell, 1983). As previously described, the immunophenotype of the IELs in RCD I and RCD II is distinct.
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Enteropathy-associated T-cell lymphoma includes type I cases and distinctive type II cases that, according to 2008 and 2010 World Health Organization descriptions, are T-cell receptor β+. Although T-cell receptor γδ enteropathy-associated T-cell lymphomas are reported, it is unknown if they have distinctive features and if they should be categorized as enteropathy-associated T-cell lymphoma or as a mucocutaneous γδ T-cell lymphoma. To address these questions, the clinicopathologic, immunophenotypic, molecular, and cytogenetic features of 5 γδ-enteropathy-associated T-cell lymphomas were investigated. Only 1 patient had celiac disease and had type I enteropathy-associated T-cell lymphoma, and the others fulfilled the histopathologic criteria for type II enteropathy-associated T-cell lymphoma. All lacked cutaneous involvement. A celiac disease–associated HLA type was found in the patient with CD and one of four others. All were T-cell receptor γ+, T-cell receptor δ+, βF1−, CD3+, CD7+, CD5−, CD4−, and TIA-1+ with variable staining for CD2 (3/5), CD8 (2/5), Granzyme B (1/5), and CD56 (4/5). Fluorescence in situ hybridization demonstrated 9q34 gains in 4 cases, with 9q33-34 gains by single nucleotide polymorphism in 3 of these. Single nucleotide polymorphism analysis also demonstrated gains in 5q34-q35.1/5q35.1 (4/5), 8q24 (3/5), and in 32 other regions in 3 of 5 cases. Vδ1 rearrangements were identified in 4 of 4 cases with documented clonality showing the same clone in normal-appearing distant mucosa (3/3 tested cases). Thus, γδ-enteropathy-associated T-cell lymphomas share many features with other enteropathy-associated T-cell lymphoma and are mostly of type II. Their usual nonactivated cytotoxic phenotype and Vδ1 usage are features unlike many other mucocutaneous γδ T-cell lymphomas but shared with hepatosplenic T-cell lymphoma. These findings support the conclusion that a γδ T-cell origin at extracutaneous sites does not define a specific entity.
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Celiac disease (CD) is associated with an increased risk of lymphoma. However, relatively few studies have assessed the outcome of patients diagnosed with both CD and lymphoma. We evaluated the temporal association between lymphoma and CD, along with clinical presentation, response to therapy, and prognosis.
Patients diagnosed with both CD and lymphoma were identified retrospectively in a tertiary referral center. Clinical characteristics and survival were analyzed.
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INTESTINAL LYMPHOMA ASSOCIATED WITH MALABSORPTION

Abstract

Eur. J. Cancer

Lancet

Clins Gastroent.

Lancet

Gastroenterology

Br. med. J.

Cancer, N.Y.

Dig. Dis.

Immunocytochemistry

Lab. Invest.

Archs Derm.

Am. J. clin. Path.

Am. J. Gastroent. N.Y.

J. clin. Path.