Lymphoma - Non B cell neoplasms
T/NK cell disorders
Indolent T-lymphoproliferative disease of the GI tract

Authors: Carlos Z. Murga, M.D., Lauren B. Smith, M.D. (see Authors page)

Revised: 8 July 2016, last major update July 2016

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PubMed search: Indolent T-lymphoblastic proliferation
Epidemiology
  • Very rare, few cases reported
Etiology
  • Due to activated, mature T lymphocytes
  • Majority are CD3+ / CD8+
Sites
  • Most cases involve the small intestine (Blood 2013;122:3599)
  • Involvement of oral cavity, stomach and colon also reported
Clinical Features
  • The most common presenting symptom is diarrhea, followed by abdominal pain
  • Male predominance (1.5 / 1.0)
  • Not associated with celiac sprue
  • Protracted clinical course with no progression or transformation to an aggressive lymphoma
  • A few reported cases show complete resolution after chemotherapy, although watchful waiting may be sufficient in classic cases
Case Reports
  • 28 year old man with indolent T cell lymphoma proliferation (CD3+, CD4+, TCR alpha-beta+, CD8-) involving the small intestine (Cancer 1994;73:1286)
  • 51 year old man with relapsing oral ulcers and concomitant colonic and terminal ileum ulcers; lymphoid proliferation was CD3+, CD4+/-, CD8- (Cancer 1995;75:1728)
  • Indolent T cell lymphoma (CD4+ / CD8-) presenting as multiple polypoid lesions in the stomach, duodenum, terminal ileum and colon (Gastroenterology 1996;111:778)
Micro Description
  • Expansion of the lamina propria by a dense clonal lymphocytic infiltrate; mucosa and submucosa may be involved
  • The infiltrate is nondestructive; adjacent glands are displaced and distorted
  • The infiltrate is predominantly 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, similar to small intestine or colon (usually concomitant)
  • Ki-67 proliferative index is low (~ 5%)
Micro Images

Images hosted on other servers:

Ileum

Duodenum

phospho-STAT3

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
Differential Diagnosis
  • Enteropathy associated T cell lymphoma (EATL): both classic and type II EATL show infiltrative and destructive lymphoid infiltrates; in contrast indolent T-LPD shows a non-destructive infiltrate; in EATL the adjacent non-involved intestinal mucosa shows morphological features of enteropathy including villous blunting and intraepithelial lymphocytes, but indolent T-LPD cases are not associated with celiac sprue or adjacent morphological evidence of enteropathy
  • Inflammatory bowel disease: 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