Small intestinal CD4+ T-cell lymphoma: a rare distinctive clinicopathological entity associated with prolonged survival

Svrcek, Magali; Garderet, Laurent; Sebbagh, Virginie; Rosenzwajg, Michèlle; Parc, Yann; Lagrange, Monique; Bennis, Malika; Lavergne‐Slove, Anne; Fléjou, Jean‐François; Fabiani, Bettina

doi:10.1007/s00428-007-0475-7

Cited by 28 publications

(49 citation statements)

References 6 publications

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“…All displayed similar morphologic, immunophenotypic and clinical features and manifested non recurrent genetic abnormalities, distinct from other types of primary enteric T-cell lymphomas. [20] In conjunction with prior reports,[14]–[17] our findings suggest the existence of a unique and rare subtype of PTCL not recognized in the current WHO classification, which warrants greater awareness for correct diagnosis and optimal management. [20].…”

Section: Introductionsupporting

confidence: 78%

“…A generalized increase in IELs is usually lacking. Carbonnel et al and Svrcek et al reported an absence of LELs in their cases, however LELs were observed in all our cases and also in the case reported by Zivny et al[14]–[17].…”

Section: Discussionsupporting

confidence: 58%

“…[14], [15] Thereafter, single case reports by Zivny et al and Svrcek et al also described what appear to be similar types of lymphomas (Table 3). [16], [17] The morphologic and immunophenotypic characteristics of our and previously published small intestinal CD4+ T-cell lymphomas are distinct from other primary T-cell lymphomas of the GI tract, such as EATL types I and II. The lymphocytic infiltrates in these lymphomas comprise a monotonous population of small to intermediate-sized cells that show mild pleomorphism and virtual restriction to the lamina propria.…”

Section: Discussionmentioning

confidence: 69%

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Indolent Small Intestinal CD4+ T-cell Lymphoma Is a Distinct Entity with Unique Biologic and Clinical Features

et al. 2013

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Enteropathy-associated T-cell lymphomas (EATL) are rare and generally aggressive types of peripheral T-cell lymphomas. Rare cases of primary, small intestinal CD4+ T-cell lymphomas with indolent behavior have been described, but are not well characterized. We describe morphologic, phenotypic, genomic and clinical features of 3 cases of indolent primary small intestinal CD4+ T-cell lymphomas. All patients presented with diarrhea and weight loss and were diagnosed with celiac disease refractory to a gluten free diet at referring institutions. Small intestinal biopsies showed crypt hyperplasia, villous atrophy and a dense lamina propria infiltrate of small-sized CD4+ T-cells often with CD7 downregulation or loss. Gastric and colonic involvement was also detected (n = 2 each). Persistent, clonal TCRβ gene rearrangement products were detected at multiple sites. SNP array analysis showed relative genomic stability, early in disease course, and non-recurrent genetic abnormalities, but complex changes were seen at disease transformation (n = 1). Two patients are alive with persistent disease (4.6 and 2.5 years post-diagnosis), despite immunomodulatory therapy; one died due to bowel perforation related to large cell transformation 11 years post-diagnosis. Unique pathobiologic features warrant designation of indolent small intestinal CD4+ T-cell lymphoma as a distinct entity, greater awareness of which would avoid misdiagnosis as EATL or an inflammatory disorder, especially celiac disease.

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Section: Introductionsupporting

confidence: 78%

Section: Discussionsupporting

confidence: 58%

Section: Discussionmentioning

confidence: 69%

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Indolent Small Intestinal CD4+ T-cell Lymphoma Is a Distinct Entity with Unique Biologic and Clinical Features

et al. 2013

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“…3,4 Several reports in the literature have described clonal T-cell proliferations in the GI tract with an indolent clinical course. [5][6][7][8][9][10][11][12][13] Recently, natural killer (NK) cell enteropathy was recognized as a distinctive benign lymphoproliferative disease mimicking intestinal lymphoma. 14,15 These patients do not require aggressive therapy and have a good prognosis.…”

Section: Introductionmentioning

confidence: 99%

Indolent T-cell lymphoproliferative disease of the gastrointestinal tract

Perry

Warnke

et al. 2013

Blood

169

300

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“…For example, an indolent small intestinal form of CD4+ T-cell lymphoma was recently recognized, often in relatively young males with unique biological and clinical features, including histopathological changes mimicking or initially confused with celiac disease [33,34,35,36]. In a subsequent report, the apparently heterogeneous origin of this small intestinal CD4+ T-cell lymphoma was emphasized [18].…”

Section: International Journal Of Celiac Diseasementioning

confidence: 99%

Sprue-like Intestinal Disease

Freeman¹

2016

IJCD

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Recurrent symptoms in well-established celiac disease may result in further clinical evaluation. In most, poor diet compliance is present. Other considerations include an unidentified gluten source, an erroneous initial diagnosis, another or second and superimposed cause for symptoms, or a complication, including collagenous sprue or an intestinal lymphoma. Failure to define an initial gluten-free diet response, however, suggests that celiac disease may not be present. Instead, a distinctive enteropathic process, refractory to diet restrictions, including gluten, is evident. This "sprue-like" intestinal disorder or enteropathy remains unclassified, and probably, represents a heterogeneous entity. Molecular changes suggestive of early clonal expansion of an aberrant population of intraepithelial lymphocytes may be detected in some (with or without a prior gluten-free diet response), and these changes may signify an early or "cryptic" lymphoma. Other newly recognized lymphoproliferative disorders occurring in the setting of celiac disease have been recorded, including hepatosplenic delta-gamma T-cell lymphoma, an indolent CD4+ T-cell lymphoma, particularly in younger males, and large granular lymphocytic leukemia, a possibly treatable disorder characterized by the clonal expansion of T-cells in blood and small intestinal mucosa. Studies using IL-15 blockade in a transgenic mouse model with pathologic features of a sprue-like intestinal disease has led to human clinical trials with encouraging positive results.

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Small intestinal CD4+ T-cell lymphoma: a rare distinctive clinicopathological entity associated with prolonged survival

Cited by 28 publications

References 6 publications

Indolent Small Intestinal CD4+ T-cell Lymphoma Is a Distinct Entity with Unique Biologic and Clinical Features

Indolent Small Intestinal CD4+ T-cell Lymphoma Is a Distinct Entity with Unique Biologic and Clinical Features

Indolent T-cell lymphoproliferative disease of the gastrointestinal tract

Sprue-like Intestinal Disease

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