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Enteropathy-Associated T-Cell Lymphoma
Enteropathy-Type T-Cell Lymphoma

Images

WIKIDATA, CC BY-SA 3.0

Presentation

A review of the literature on EATL is also presented. [ncbi.nlm.nih.gov]

The majority of EATLs present as large cell lymphoma CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text]; however, they also present as small cell lymphoma CD[Formula: See Text [doi.org]

Entire Body System

  • Fatigue

    ICD-9 Codes Corresponding ICD-10 Codes C84.5 Other and unspecified T-cell lymphomas Corresponding ICD-10-CM Codes (U.S. only) C86.2 Enteropathy-type (intestinal) T-cell lymphoma (effective October 01, 2015) Signs and Symptoms Drenching night sweats Fatigue [seer.cancer.gov]

    It may include the following modalities: Chemotherapy: This approach uses a combination of drugs to kill the cancerous cells and can be used in patients, for all stages of the tumor There can be severe side effects including fatigue, nausea, hair loss [dovemed.com]

    In addition, if you ever develop any symptoms of non-Hodgkin lymphoma (which can include swollen lymph nodes, fatigue, unexplained weight loss, fever and night sweats), you should talk to your doctor immediately, and make sure she knows the connection [verywell.com]

    Unlike EATL, it often occurs in celiac disease patients who, while otherwise well-maintained on a gluten-free diet, have vague gastrointestinal symptoms such as fatigue, malaise, and weight loss. [en.wikipedia.org]

  • Painless Jaundice

    We report a patient with a 13-year history of celiac sprue presenting with painless jaundice and a Courvoisier gallbladder. [ncbi.nlm.nih.gov]

Gastrointestinal

  • Abdominal Pain

    A 64-year-old woman was referred with a short history of abdominal pain, anorexia and weight loss. Investigations revealed a probable enteropathy associated T-cell lymphoma (EATL). [ncbi.nlm.nih.gov]

  • Severe Abdominal Pain

    She was admitted because of sudden onset of severe abdominal pain. [ncbi.nlm.nih.gov]

    Later that evening, patient developed severe abdominal pain, which did not respond to the usual symptomatic treatment. He now developed peritoneal signs and rapidly evolved into sepsis with hypotension, azotemia, and acidosis. [jmedscindmc.com]

Face, Head & Neck

  • Facial Mass

    Extranodal NK/T cell lymphoma is a lymphoma of natural killer (NK) or T cell lineage that most commonly presents with a facial mass. A small percentage of patients may present with gastrointestinal involvement. [diagnosticpathology.biomedcentral.com]

Workup

The suspicion of EATL should lead to an extensive diagnostic workup in which magnetic resonance enteroclysis, positron emission tomography scan, and histologic identification of lesions represent the best options. [ncbi.nlm.nih.gov]

Some patients with no history of coeliac disease present with symptoms and/or signs of a small intestinal lymphoma but on diagnostic workup are found to have coeliac disease. [en.wikipedia.org]

X-Ray

  • Pneumoperitoneum

    The chest radiography showed pneumoperitoneum, and an exploratory laparotomy revealed perforation of the small bowel. An advanced stage (Ann Arbor IV B/Lugano IIE2B) EATL type II was diagnosed. [revistas.usp.br]

Ultrasound

  • Ovarian Mass

    We report a case of EATL in a 49-year-old female presenting as bilateral ovarian masses. The morphology and immunophenotypic features were compatible with monomorphic variant of EATL. [ncbi.nlm.nih.gov]

Gastroscopy

  • Gastric Lesion

    The gastric EATL should be distinguished from other gastric lesions, such as peptic ulcer, poorly-differentiated adenocarcinoma and other types of lymphoma. [ncbi.nlm.nih.gov]

Colonoscopy

  • Multiple Ulcerations

    […] to avoid perforation ( Case Rep Oncol 2009;2:36 ) Postulated normal counterpart Intraepithelial T cells of the intestine Gross description Multiple ulcerated mucosal masses, also single / multiple ulcers or large exophytic mass Gross images Images hosted [pathologyoutlines.com]

    EATL usually affects the jejunum and grossly (visible to the eye) appears as multiple ulcers causing circumferential thickening of affected bowel wall without the formation of definite tumor masses most commonly in the proximal small bowel. [glutenfreeworks.com]

    The resected jejunal and ileal segments showed multiple irregular, variable-sized ulcers, the largest measuring 3.5 cm × 2 cm. Some ulcers were deep perforating. Microscopic examination of the ulcers showed florid mixed inflammatory infiltrate. [indianjcancer.com]

HLA Type

  • HLA-DR3

    (See Coeliac Disease, HLA-DQ, HLA DR3-DQ2 ) A DQ isoform that appears to be responsible for EATL in the overwhelming number of cases is highly effective at presenting a proteolytically protected region of α2-glaidin to T-cells, constant over-stimulation [ourmed.org]

Treatment

There are no standardized treatment protocols, and the optimal therapy for EATL remains unclear. The primary step of treatment consists of local debulking, preferably as early as possible after EATL diagnosis. [ncbi.nlm.nih.gov]

Prognosis

High-dose chemotherapy with autotransplantion seems to only improve the prognosis in localised forms. Allogeneic bone marrow transplantation was not evaluated. [ncbi.nlm.nih.gov]

It is the most common primary gastrointestinal T-cell lymphoma and is characterized by its aggressive course and poor prognosis. [glutenfreeworks.com]

Etiology

Inflammatory bowel disease, celiac disease, immunosuppression, and infectious etiologies, such as Epstein-Barr and human T-lymphotropic viruses, have been reported as risk factors, but no direct causal link has been established. [ncbi.nlm.nih.gov]

Source: Aliment Pharmacol Ther. 2010 Dec;32(11-12):1392-7. celiac disease celiac disease etiology lymphocytic duodenosis prospective study Read more... 1 comment 10,080 views [celiac.com]

Higher frequency in areas with high prevalence of celiac disease (northern Europe) Monomorphic variant (type II EATL): 10% - 20% of cases of EATL, broader geographic distribution and sporadic occurrence in areas where celiac disease is rare (Asia) Etiology [pathologyoutlines.com]

Etiologies and predictors of diagnosis in nonresponsive celiac disease. Clin Gastroenterol Hepatol. 2007; 5 (4): 445-450. Verbeek WH, von Blomberg BM, Coupe VM, Daum S, Mulder CJ, Schreurs MW. [medigraphic.com]

Epidemiology

The epidemiology, presentation, diagnosis, management and prognosis of this rare condition are discussed. [ncbi.nlm.nih.gov]

Pathophysiology

Consecutive pathophysiological and immunohistochemical analyses confirmed the diagnosis of EATCL: CD45RO+, CD43+, CD3+. The revision of renal pathophysiology substantiated the diagnosis. [ncbi.nlm.nih.gov]

Pathophysiology Type 1 Arises in a background of celiac disease. [cancertherapyadvisor.com]

Declarations Authors’ Affiliations (1) Department of Pathologym, the First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, 110001, China (2) Institute of Pathology and Pathophysiology, China Medical University [diagnosticpathology.biomedcentral.com]

Prevention

Moreover, the high incidence of severe postsurgical complications and the poor nutritional and immunological conditions lead to progressive deterioration of these patients, preventing the use of an adequate and effective treatment. [ncbi.nlm.nih.gov]

There is no satisfactory treatment for EATL, the only possibility of preventing EATL development in RCD being autologous bone marrow transplantation. [doi.org]

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