Lymphoma & related disorders

Mature T/NK cell disorders

Intestinal

Indolent T lymphoproliferative disease of the GI tract


Editorial Board Member: Elizabeth Courville, M.D.
Deputy Editor-in-Chief: Genevieve M. Crane, M.D., Ph.D.
Anamarija M. Perry, M.D.

Topic Completed: 22 July 2021

Minor changes: 22 July 2021

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PubMed search: Indolent T cell lymphoproliferative disease of the GI tract

Anamarija M. Perry, M.D.
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Cite this page: Perry AM. Indolent T lymphoproliferative disease of the GI tract. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonBindolentTcell.html. Accessed October 28th, 2021.
Definition / general
  • Clonal T cell lymphoproliferative disorder (T-LPD) that involves the mucosa of gastrointestinal (GI) tract
Essential features
  • Rare, clinically indolent clonal T cell lymphoproliferative disease confined to the GI tract
  • Can involve all GI sites, with small intestine and colon being most commonly involved
  • Most patients are middle aged men, frequently presenting with diarrhea
  • Nondestructive infiltrate of small, monotonous lymphoid cells that can be CD4+ / CD8- or CD4- / CD8+
  • Poor response to chemotherapy; chronic course with frequent relapses and prolonged survival
ICD coding
  • ICD-O: 9702/1 - indolent T cell lymphoproliferative disorder of the gastrointestinal tract
Epidemiology
Sites
  • Most commonly involves small intestine and colon but any GI site can be involved (Blood 2013;122:3599)
  • Rarely associated with regional (abdominal) lymphadenopathy
  • Occasional reports of bone marrow involvement
Etiology
  • Unknown
Clinical features
  • Most common presenting symptoms are abdominal pain and diarrhea (Blood 2013;122:3599)
  • Other symptoms / signs include vomiting, weight loss, signs of malabsorption
  • Disease can clinically mimic inflammatory bowel disease
  • Some patients had a history of Crohn's disease
  • Chronic course with frequent relapses
  • Prolonged survival with persistent disease
  • 4 reported cases in the literature of progression to aggressive T cell lymphoma, with poor outcome (Int J Surg Pathol 2019;27:102)
Diagnosis
  • Biopsy of affected GI site
Prognostic factors
  • No specific prognostic factors identified
Case reports
Treatment
  • Poor response to conventional chemotherapy and immunotherapy; so far, there is no successful treatment
Microscopic (histologic) description
  • Dense lymphoid infiltrate in the lamina propria / mucosa, occasionally extending into the submucosa (Blood 2013;122:3599)
  • Infiltrate is nondestructive; adjacent glands are displaced and distorted
  • Infiltrate is composed of monomorphic small lymphoid cells with scant pale cytoplasm, slightly irregular nuclear contours, mature chromatin, inconspicuous nucleoli
  • Cases with oral involvement manifest as ulcers with an underlying lymphocytic infiltrate
  • Typically no necrosis, ulceration or angiocentricity / angiodestruction (Blood 2013;122:3599)
Microscopic (histologic) images

Contributed by Anamarija M. Perry, M.D.
Colon with indolent T-LPD Colon with indolent T-LPD

Colon with indolent T-LPD

Infiltration of lamina propria

Infiltration of lamina propria

Atypical infiltrate of indolent T-LPD

Atypical infiltrate of indolent
T-LPD


CD8 immunohistochemical stain

CD8 IHC stain

CD4 immunohistochemical stain

CD4 IHC stain

TIA1 immunohistochemical stain

TIA1 IHC stain

Virtual slides

Images hosted on other servers:
Colonic mucosa with indolent T-LPD

Colonic mucosa with indolent
T-LPD

Positive stains
Negative stains
Flow cytometry description
  • Immunophenotype similar to mature T lymphocytes
  • Aberrant loss of CD7 is rarely seen
Molecular / cytogenetics description
  • Clonal TCR gene rearrangements present by PCR testing
  • t(9;17)(p24.1;q21.2) STAT3-JAK2 fusion found as recurrent abnormality, mainly in CD4+ cases (Blood 2018;131:2262)
  • Genetic alterations are common, including recurrent mutations and novel rearrangements, often involving JAK-STAT pathway genes (Haematologica 2020;105:1895)
Differential diagnosis
  • Intestinal T cell lymphoma:
  • Celiac sprue:
    • In cases of indolent T-LPD that involve duodenum, differential diagnosis includes celiac sprue
    • Distribution of lymphocytes in indolent T-LPD is not characteristic for celiac sprue (no clustering at the tip)
    • There are no other histologic features of sprue (i.e. villous atrophy, hyperplastic and elongated crypts, increase in plasma cells in lamina propria)
  • Inflammatory bowel disease (IBD):
    • Crypt distortion is present in indolent T-LPD
    • However, other morphological features that are usually present in IBD are absent, including cryptitis, crypt abscesses, basal plasmacytosis and Paneth cell hyperplasia
Board review style question #1

A 52 year old man presents with a 10 year history of intermittent abdominal pain and diarrhea, which usually resolves in several weeks without treatment. Colonoscopy revealed erythematous irregular mucosa throughout the entire length of colon. Biopsy was taken and infiltrate is demonstrated in the image. The infiltrate involves mainly lamina propria, it is nondestructive and is positive for CD8 and TIA1 immunohistochemical stains. Molecular study was performed and showed clonal T cell gene rearrangement. Which of the following is true about this disease?

  1. Infiltrate is destructive and frequently involves the entire thickness of bowel wall
  2. It is successfully treated with CHOP chemotherapy
  3. Most cases are CD4- / CD8-
  4. Oral cavity is the most common site of involvement
  5. Subset of cases show STAT3-JAK2 fusion
Board review style answer #1
E. Subset of cases show STAT3-JAK2 fusion

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Reference: Indolent T lymphoproliferative disease of the GI tract
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