Enteropathy-associated T-cell lymphoma

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Enteropathy-associated T-cell lymphoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Synonyms and keywords: Enteropathy-type T-cell lymphoma; Intestinal T-cell lymphoma; EATL; ETTL

Overview

Enteropathy-associated T-cell Lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell non-hodgkin lymphoma that affects the small intestine, it is composed of large lymphoid cells. Enteropathy-associated T-cell lymphoma has two subtypes, type I enteropathy-associated T-cell lymphoma which has a strong association with celiac disease and it is more common in western countries and type II enteropathy-associated T-cell lymphoma which is mostly found among the Asian population. Genes involved in the pathogenesis of this disease include 8q24, T-cell receptor (TCR) beta and gamma, and 16q genes. On gross pathology, multiple intestinal ulcers are characteristic findings of EATL. On microscopic histopathological analysis, monotonous cells, round or angulated vesicular nuclei, and prominent nucleoli are characteristic findings of enteropathy-associated T-cell lymphoma. There are no established causes for enteropathy-associated T-cell lymphoma. EATL must be differentiated from other diseases such as peptic ulcer, poorly-differentiated adenocarcinoma, MALT lymphoma, diffuse large B cell lymphoma, and mantle cell lymphoma. The incidence of enteropathy-associated T-cell lymphoma is very low worldwide. There are no established risk factors for enteropathy-associated T-cell lymphoma. Common complications of enteropathy-associated T-cell lymphoma include ulcer, obstruction, and perforation of small intestine. Prognosis is generally poor. According to the Lugano classification, there are four stages of enteropathy-associated T-cell lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of enteropathy-associated T-cell lymphoma include fever, weight loss, skin rash, night sweats, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of enteropathy-associated T-cell lymphoma include fever, rash, ulcer, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy. Lymph node or endoscopic tissue biopsy is diagnostic of enteropathy-associated T-cell lymphoma. CT scan may be helpful in the diagnosis of enteropathy-associated T-cell lymphoma. Findings on CT scan suggestive of enteropathy-associated T-cell lymphoma include bowel wall thickening, mesenteric fat infiltration, mesentric lymph node cavitation, intussusception, and small-sized spleen. Other diagnostic studies for enteropathy-associated T-cell lymphoma include bone marrow aspiration and bone marrow biopsy. There is no treatment, the mainstay of therapy is supportive care. The optimal therapy depends on the extent and the location of the lymphoma in the small intestine. Surgery is not the first ­line treatment option for patients with enteropathy-associated T-cell lymphoma. Local debulking is usually reserved for patients with tumor masses with a high risk of obstruction, hemorrhage, and perforation.

Historical Perspective

[Disease name] was first discovered by [name of scientist], a [nationality + occupation], in [year]/during/following [event].

The association between [important risk factor/cause] and [disease name] was made in/during [year/event].

In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].

In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].

There have been several outbreaks of [disease name], including -----.

In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • Type I enteropathy-associated T-cell lymphoma
  • Type II enteropathy-associated T-cell lymphoma
Enteropathy-associated T-cell lymphoma classification[1][3][4]
Name Description
Classical enteropathy-associated T-cell lymphoma (Type I Enteropathy-associated T-cell lymphoma)
  • Covers 80-90% of all enteropathy-associated T-cell lymphoma cases.
  • Is usually associated with refractory celiac disease that is sometimes accompanied by intestinal ulceration (ulcerative jejunitis)
  • Most show adult onset disease or are diagnosed as having celiac disease in the same clinical episode in which the lymphoma is diagnosed.
  • Frequently has large-cell or pleomorphic cytology.
  • Seldom expresses CD8 and CD56.
Monomorphic enteropathy-associated T-cell lymphoma (Type II enteropathy-associated T-cell lymphoma)
  • Encountered in Asia and other regions where celiac disease is rare.
  • Is sporadic, seldom associated with celiac disease.
  • Covers 10-20% of all enteropathy-associated T-cell lymphoma cases.
  • Is characterized by monomorphic cytology.
  • Frequent expression of CD8 and CD56.
  • The tumor forms an ulcerating mucosal mass that invades the wall of the intestine.
  • Poor prognosis, death occurs from abdominal complications such as small bowel obstruction, perforation.

Pathophysiology

  • Enteropathy-associated T-cell Lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell lymphoma that affects the small intestine. [5]
  • Frequent activating mutations in the JAK-STAT pathway in EATL suggests that deregulation of cytokine signaling is the early event in lymphomagenesis.
  • Intraepithelial T cells are presumed to be the cell of origin of EATL (and RCD II).
  • Variable degrees of transformations can be seen on histopathology of this tumor, but usually presents as large lymphoid cells.[3]
  • These cancerous T-cells are a possible consequence of either refractory cases of celiac disease or chronic untreated celiac disease patients.
  • Most commonly occurs in the jejunum or ileum.
  • SETD2 was found to be the most often silenced gene in EATL according to studies.[6]
  • The JAK-STAT pathway is the most frequently mutated pathway. [6]
  • Mutations in KRAS, TP53, and TERT Type I EATL and type II EATL (monomorphic intestinal T cell lymphoma) identified as well which have overlapping genetic alterations.

Causes

  • There are no established causes for enteropathy-associated T-cell lymphoma.

Differentiating ((Enteropathy-associated T cell lymphoma)) from Other Diseases

Enteropathy-associated T-cell lymphoma must be differentiated from other diseases such as:[1][7]

Epidemiology and Demographics

Risk Factors

  • There are no established risk factors for enteropathy-associated T cell lymphoma.

Screening

  • There is insufficient evidence to recommend routine screening for enteropathy-associated T-cell lymphoma.

Natural History, Complications, and Prognosis

  • Enteropathy-associated T-cell lymphoma is usually a fast-growing (aggressive) lymphoma.[9]
  • It is associated with celiac disease (sprue).
  • Most adults are diagnosed with celiac disease at the same time as their lymphoma or shortly before their lymphoma is diagnosed.
  • Enteropathy-associated T-cell lymphoma may spread to the liver, spleen, lymph nodes, gallbladder, stomach, colon or skin.

Diagnosis

Diagnostic Study of Choice

  • Endoscopic biopsy is the diagnostic study of choice for enteropathy-associated T cell lymphoma, though CT scan and other imaging studies can be helpful.[10]

History and Symptoms

Physical Examination

[3]==

Vitals

Skin

HEENT

Thorax

Abdomen

Extremities

Laboratory Findings

An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].

OR

Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

OR

[Test] is usually normal among patients with [disease name].

OR

Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].

OR

There are no diagnostic laboratory findings associated with [disease name].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgical intervention is not recommended for the management of [disease name].

OR

Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

OR

The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of [disease name].

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

OR

[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

References

  1. 1.0 1.1 1.2 Enteropathy-associated T-cell lymphoma. BioMed Central. http://diagnosticpathology.biomedcentral.com/articles/10.1186/1746-1596-7-172. Accessed on January 28, 2016
  2. V G, Kudva R, Amprayil AJ (October 2014). "Enteropathy associated T cell lymphoma - a case report of an uncommon extranodal T cell lymphoma". J Clin Diagn Res. 8 (10): FD10–2. doi:10.7860/JCDR/2014/9740.4999. PMC 4253173. PMID 25478355.
  3. 3.0 3.1 3.2 3.3 Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 26, 2016
  4. Hussain N, Hussain F, Chatterjee T, Upalakalin JN, Lynch T (2018). "An unexpected deterrent in diagnosing refractory celiac disease and enteropathy-associated T-cell lymphoma: a gluten-free diet". J Community Hosp Intern Med Perspect. 8 (4): 233–236. doi:10.1080/20009666.2018.1483693. PMC 6116147. PMID 30181834.
  5. Bautista-Quach MA, Ake CD, Chen M, Wang J (September 2012). "Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features". J Gastrointest Oncol. 3 (3): 209–25. doi:10.3978/j.issn.2078-6891.2012.024. PMC 3418529. PMID 22943012.
  6. 6.0 6.1 Moffitt AB, Ondrejka SL, McKinney M, Rempel RE, Goodlad JR, Teh CH, Leppa S, Mannisto S, Kovanen PE, Tse E, Au-Yeung R, Kwong YL, Srivastava G, Iqbal J, Yu J, Naresh K, Villa D, Gascoyne RD, Said J, Czader MB, Chadburn A, Richards KL, Rajagopalan D, Davis NS, Smith EC, Palus BC, Tzeng TJ, Healy JA, Lugar PL, Datta J, Love C, Levy S, Dunson DB, Zhuang Y, Hsi ED, Dave SS (May 2017). "Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2". J. Exp. Med. 214 (5): 1371–1386. doi:10.1084/jem.20160894. PMC 5413324. PMID 28424246. Vancouver style error: initials (help)
  7. Isaacson P, Wright DH (January 1978). "Intestinal lymphoma associated with malabsorption". Lancet. 1 (8055): 67–70. PMID 74567.
  8. Verbeek WH, Van De Water JM, Al-Toma A, Oudejans JJ, Mulder CJ, Coupé VM (2008). "Incidence of enteropathy-associated T-cell lymphoma: a nation-wide study of a population-based registry in The Netherlands". Scand. J. Gastroenterol. 43 (11): 1322–8. doi:10.1080/00365520802240222. PMID 18618372.
  9. Enteropathy-associated T-cell lymphoma . Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/enteropathy-associated-t-cell-lymphoma/?region=on Accessed on January 27, 2016
  10. Di Sabatino, A.; Biagi, F.; Gobbi, P. G.; Corazza, G. R. (2012). "How I treat enteropathy-associated T-cell lymphoma". Blood. 119 (11): 2458–2468. doi:10.1182/blood-2011-10-385559. ISSN 0006-4971.


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