Dr  Sampurna Roy  MD

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The presence of a monoclonal T-cell population in nonlymphomatous enteropathic small intestinal mucosa has been described in Enteropathy associated  T-cell lymphoma , ulcerative jejunitis, and nonresponsive coeliac disease.

Visit: Malabsorption syndrome ; Coeliac Disease ;An approach to evaluation of small intestinal biopsy

Enteropathy associated  T-cell lymphoma (EATL) of the small intestine is a well-documented complication of coeliac disease.

It is present in patients with longstanding coeliac disease ( gluten sensitive enteropathy ) but more often follows a short history of adult coeliac disease.

Patients are relatively young adults (30-40yrs).

Lymphoma is suspected when there is a reversal to response to gluten withdrawal and patients complain of fever, weight loss and abdominal pain.

Extensive sampling of the lesion, careful evaluation of the biopsy and a close follow up is advised as often it can be very difficult to make the diagnosis with certainty.

It has been suggested on the basis of previous molecular analysis, and the immunophenotypic findings that the monoclonal intraepithelial lymphocytes in patients with complications of coeliac disease are neoplastic, although they are not cytologically abnormal and they do not form tumour masses.

Ulcerative jejunitis (UJ) and Enteropathy-associated T-cell lymphoma (EATL) are closely related conditions both associated with celiac disease. Benign-appearing inflammatory ulcers are seen in both, which has led to the suggestion that UJ is a manifestation of EATL. It has been shown that T-cell monoclonality is a feature of the ulcers in both UJ and EATL and the same clone is present in EATL and its associated inflammatory ulcers and in UJ and subsequently developing lymphoma.

Enteropathy-associated T-cell lymphoma is an aggressive disease with poor prognosis but strict adherence to a gluten free diet may prevent its occurrence.

Normal histology of the small intestine for anatomic pathologists

Microscopic features: Image Link(blood journal)

- Ulcers associated with sprue-like mucosa. Fissuring ulcers are noted mimicking Crohn's disease.

- Severe villous atrophy and crypt hyperplasia.

- Dense infiltration of the epithelium by lymphocytes.

- Initially there is a mixed cellular infiltrate.

- Gradual increase in atypical large lymphoid cells. Binucleate or multinucleate cells are noted.

- Eventually there is transformation into full blown lymphoma.

- CD3 (+), CD4(-), CD8 (+/-), UCHL1 (+), TIA-1 (+), CD103 (+).

- Some cases are associated with Epstein-Barr virus.

- If an intestinal lymphoma morphologically looks like an anaplastic large cell lymphoma and is CD30+, it should not be called an anaplastic large cell lymphoma if there are features consistent with enteropathy-type T-cell lymphoma.

Abstracts: A rare case of enteropathy-associated T-cell lymphoma presenting as acute renal failure.World J Gastroenterol. 2006 Apr 14;12(14):2301-4. Hematological manifestations of celiac disease.Blood. 2006 Sep 14; What is the role of celiac disease in enteropathy-type intestinal lymphoma? A retrospective study of nine cases.Acta Gastroenterol Belg. 2005;68(4):419-23. Association of celiac disease and intestinal lymphomas and other cancers. Gastroenterology. 2005 Apr;128(4 Suppl 1):S79-86 Frequency of clonal intraepithelial T lymphocyte proliferations in enteropathy-type intestinal T cell lymphoma, coeliac disease, and refractory sprue.Gut. 2001 Dec;49(6):804-12. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group.Lancet. 2000 Jul 15;356(9225):203-8 Cytotoxic T-cell lymphoma diffusely involving the entire gastrointestinal tract associated with Epstein-Barr virus and tubercle bacilli infection. Int J Hematol. 2000 Jun;71(4):379-84 Mucosal intra-epithelial lymphocytes in enteropathy-associated T-cell lymphoma, ulcerative jejunitis, and refractory celiac disease constitute a neoplastic population.Blood. 1999 Jul 1;94(1):260-4. Most CD56+ intestinal lymphomas are CD8+ CD5 T-cell lymphomas of monomorphic small to medium size histology. Am J Pathol 153:1483, 1998 Are complicated forms of celiac disease cryptic T-cell lymphomas? Blood 92:3879, 1998 Enteropathy associated T-cell lymphomas have a cytotoxic T-cell phenotype. Histopathology 31:313, 1997 Molecular analysis of T-cell clonality in ulcerative jejunitis and enter opathy-associated T-cell lymphoma.Am J Pathol. 1997 Aug;151(2):493-8. Custom Search                              Your Ad Here Pathopedia-India.com: Contents ; Introduction of Pathology ; An outline of Diagnostic Techniques available in Pathology ; Cellular Injury ; Diagram showing Structural Changes in Reversible and Irreversible Cell Injury ; Autolysis; Heterolysis ; Necrosis; Coagulation (Coagulative) necrosis ; Caseative (Caseous) necrosis ; Liquefaction necrosis ; Fat necrosis ; Fibrinoid necrosis ; Apoptosis ; Gangrene ; Hyaline Change ; Atrophy ; Hypertrophy ; Hyperplasia ; Metaplasia ; Aplasia ; Hypoplasia ;Cellular Accumulations ; Accumulation of Glycogen, complex lipids and carbohydrates ; Pigments ; Melanin ; Pigments derived from Hemoproteins; Hemosiderin and Hemosiderosis ; Primary Hemochromatosis ; Hematin; Bilirubin; Lipofuscin; Mineral Dusts ; Silica ; Urate ; Amyloid ; Inflammation ; Inflammatory cells in acute and chronic inflammation ; Acute Inflammation; Types of Acute Inflammation; Chemical Mediators ; Chronic Inflammation; Wound Healing ; Circulatory Anatomy, Physiology and Regulation; Normal Fluid Balance; Edema; Morphology of Edema; Diagram showing Capillary System and Mechanisms of Edema Formation; Hyperemia and Congestion; Hemostasis and Thrombosis; Embolism; Fat Embolism; Air Embolism ; Decompression Sickness ; Amniotic Fluid Embolism ; Diagram showing Sources of Arterial Emboli ; Diagram showing Sources of Venous Emboli ; Infarction ; Diagram showing common sites of Systemic Infarction  from Arterial Emboli; Shock; Pathology of Shock; Diagram showing Complications of Shock; Hemorrhage; Glycogen Storage Diseases(Part I ) ;Glycogen Storage Diseases ( Part II ); Alkaptonuria; Neurofibromatosis; Down Syndromie (Trisomy 21); Edward Syndrome (Trisomy 18); Patau Syndrome (Trisomy 13); Chromosome 22q11 Deletion Syndrome: (Also known as DiGeorge or Velo-Cardio-Facial Syndrome).