| 词条 | Enteropathy-associated T-cell lymphoma |
| 释义 |
| name = Enteropathy-associated T-cell lymphoma | synonyms = | image = Enteropathy-associated T cell lymphoma - low mag.jpg | caption = Micrograph of enteropathy-associated T cell lymphoma (upper right of image). H&E stain. | pronounce = | field = | width = 150 | | symptoms = | complications = Bowel obstructions and perforations | onset = | duration = | types = | causes = Complication of Celiac disease | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} 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. It is the most common primary gastrointestinal T-cell lymphoma, arising from the T cells that are found between the cells that line the small intestinal (brush border cells or small intestinal epithelial cells).[1] These cancerous T-cells are a possible consequence of refractory cases of coeliac disease or in chronic, untreated cases in genetically susceptible individuals. In 2008, the World Health Organization defined a specific type of lymphoma, enteropathy-associated T cell lymphoma (EATL), as having two different types: EATL type I, a lymphoma occurring in patients with the chronic, autoimmune GI tract disorder, celiac disease, and EATL type II, a similar bowel lymphoma that was not associated with celiac disease. However, subsequent studies found significant clinical, pathologic, and pathophysiological differences between these two types of lymphoma. Consequently, the World Health Organization (2016) redefined these lymphomas as separate entities, terming the celiac disease-associated lymphoma as enteropathy-associted T cell lymphoma and the lymphoma not associated with celiac disease as monomorphic epitheliotropic intestinal T cell lymphoma (MEITL).[2] MEITL is only 1/5 to 1/10 as common as EATL.[3] The Organization (2016} also termed a third type of intestinal T cell lymphoma that could not be classified as EATL or MEITL as intestinal T cell lymphoma, not otherwise specified (ITCL-NOS).[4] Accordingly, monomorphic epitheliotropic intestinal T cell lymphoma is considered on a new, separate Wikipedia page. ClassificationEATL can be classified as an extranodal peripheral T cell lymphoma, a category it shares with hepatosplenic T cell lymphoma and panniculitic T cell lymphoma. It can be further classified in type I and II EATL.[5] GeneticsEnteropathy associated T-cell lymphoma (EATL) is environmentally induced as a result of the consumption of Triticeae glutens (e.g. wheat gluten). In gluten-sensitive individuals with EATL, 68% are homozygotes of the DQB1{{HQBA|02}} subtype at the HLA-DQB1 locus.[5] (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-gliadin to T-cells, constant over-stimulation of T-cell eventually results in neoplastic growth.[6] EATL typically appears after the 4th decade of life, within 3 years of coeliac disease diagnosis or in undiagnosed coeliacs.[7][8] In treated coeliacs, EATL may be preceded by refractory coeliac disease 1(RCD1) or, prominently, refractory celiac disease 2 (RCD2), in which EATL is a frequent outcome.[9] Refractory coeliac disease is no longer favorably responsive to wheat-gluten abstinence. Beyond the RCD1 stage, many drugs are not effective, and undetected coeliac disease leading to de novo EATL generally has a poor outcome. The genetic association with celiac disease and HLA loci defines type I EATL. Type II doesn´t show these associations and frequently presents with bulky disease. Early recognition of coeliac disease, particularly with a focus on DQ2 homozygotes and in affected family members, is the only effective prevention, though bone marrow transplant was suggested as a treatment during early RCD2.[10] StagingBone marrow involvement is rare in this disease.{{citation needed|date=May 2013}} TreatmentIn certain eligible patients, a conditioning regimen of high-dose chemotherapy followed by an autologous stem cell transplant may be used to extend a period of first complete remission.[11] Likewise, a recent study suggests that high dose therapy and autologous stem cell transplantation results in favorable outcomes for elderly patients with Non-Hodgkin's Lymphoma.[12] PrognosisAccording to the Peripheral T-Cell Lymphoma Project, median overall survival is ten months, while median failure-free survival is only six months . The peripheral index for T-cell lymphoma is useful in defining prognosis for enteropathy-associated T-cell lymphoma. Among the most influential prognostic factors is bulky disease, defined by a tumor mass greater than 5 cm.[5] Autologous stem cell transplantation is feasible for selected patients with enteropathy-associated T-cell lymphoma and can yield durable disease control in a significant proportion of these patients. One study found a trend for better survival in patients transplanted in first complete or partial remission at four years (66% vs. 36%; P = .062).[11]EpidemiologyEATL is most frequent in Europe, where it represents 9.4% of all peripheral T cell lymphomas. Association with celiac disease is consistently demonstrated in only 30% of patients. The global incidence of this lymphoma is rare, being about 0.5 to 1 per million.[13] See also
References1. ^{{cite journal |author=Isaacson PG |title=Gastrointestinal lymphoma |journal=Hum. Pathol. |volume=25 |issue=10 |pages=1020–9 |date=October 1994 |pmid=7927306 |doi=10.1016/0046-8177(94)90060-4}} 2. ^{{cite journal | vauthors = Chander U, Leeman-Neill RJ, Bhagat G | title = Pathogenesis of Enteropathy-Associated T Cell Lymphoma | journal = Current Hematologic Malignancy Reports | volume = 13 | issue = 4 | pages = 308–317 | date = August 2018 | pmid = 29943210 | doi = 10.1007/s11899-018-0459-5 | url = }} 3. ^{{cite journal | vauthors = Ondrejka S, Jagadeesh D | title = Enteropathy-Associated T-Cell Lymphoma | journal = Current Hematologic Malignancy Reports | volume = 11 | issue = 6 | pages = 504–513 | date = December 2016 | pmid = 27900603 | doi = 10.1007/s11899-016-0357-7 | url = }} 4. ^{{cite journal | vauthors = Matutes E | title = The 2017 WHO update on mature T- and natural killer (NK) cell neoplasms | journal = International Journal of Laboratory Hematology | volume = 40 Suppl 1 | issue = | pages = 97–103 | date = May 2018 | pmid = 29741263 | doi = 10.1111/ijlh.12817 | url = }} 5. ^{{cite journal |vauthors=Al-Toma A, Verbeek WH, Hadithi M, von Blomberg BM, Mulder CJ |title=Survival in Refractory Coeliac Disease and Enteropathy associated T cell Lymphoma: Retrospective evaluation of single centre experience |journal= Gut|volume= 56|issue= 10| pages = 1373–8|year=2007 |pmid=17470479 |pmc=2000250 |doi=10.1136/gut.2006.114512}} 6. ^{{cite journal |vauthors=Jores RD, Frau F, Cucca F, etal | title = HLA-DQB1*0201 homozygosis predisposes to severe intestinal damage in celiac disease | journal = Scand. J. Gastroenterol. | volume = 42 | issue = 1 | pages = 48–53 | year = 2007 | pmid = 17190762 | doi = 10.1080/00365520600789859}} 7. ^{{cite journal |vauthors=Al-Toma A, Goerres MS, Meijer JW, etal | title = Cladribine therapy in refractory celiac disease with aberrant T cells | journal = Clin. Gastroenterol. Hepatol. | volume = 4 | issue = 11 | pages = 1322–7; quiz 1300 | year = 2006 | pmid = 16979946 | doi = 10.1016/j.cgh.2006.07.007}} 8. ^{{cite journal |vauthors=Al-Toma A, Goerres MS, Meijer JW, Peña AS, Crusius JB, Mulder CJ | title = Human leukocyte antigen-DQ2 homozygosity and the development of refractory celiac disease and enteropathy-associated T-cell lymphoma | journal = Clin. Gastroenterol. Hepatol. | volume = 4 | issue = 3 | pages = 315–9 | year = 2006 | pmid = 16527694 | doi = 10.1016/j.cgh.2005.12.011}} 9. ^{{cite journal |vauthors=Al-Toma A, Verbeek WH, Mulder CJ | title = Update on the management of refractory coeliac disease | journal = Journal of Gastrointestinal and Liver Diseases | volume = 16 | issue = 1 | pages = 57–63 | year = 2007 | pmid = 17410290 | doi = }} 10. ^{{cite journal |vauthors=Meijer JW, Mulder CJ, Goerres MG, Boot H, Schweizer JJ |title=Coeliac disease and (extra)intestinal T-cell lymphomas: definition, diagnosis and treatment |journal=Scand. J. Gastroenterol. Suppl. |volume= 39|issue=241 |pages=78–84 |year=2004 |pmid=15696854 |doi= 10.1080/00855920410014605|url=}} 11. ^1 {{Cite journal | pmid = 23361910| year = 2013| author1 = Jantunen| first1 = E| title = Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: A retrospective study by the EBMT| journal = Blood| volume = 121| issue = 13| pages = 2529–32| last2 = Boumendil| first2 = A| last3 = Finel| first3 = H| last4 = Luan| first4 = J. J.| last5 = Johnson| first5 = P| last6 = Rambaldi| first6 = A| last7 = Haynes| first7 = A| last8 = Duchosal| first8 = M. A.| last9 = Bethge| first9 = W| last10 = Biron| first10 = P| last11 = Carlson| first11 = K| last12 = Craddock| first12 = C| last13 = Rudin| first13 = C| last14 = Finke| first14 = J| last15 = Salles| first15 = G| last16 = Kroschinsky| first16 = F| last17 = Sureda| first17 = A| last18 = Dreger| first18 = P| author19 = Lymphoma Working Party of the EBMT| doi = 10.1182/blood-2012-11-466839}} 12. ^https://synapse.mskcc.org/synapse/works/39346 13. ^1 2 {{cite journal |vauthors=Delabie J, etal |title=Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project|journal=Blood |volume=118|issue=148|pages= 148–55|date=July 2011 |doi=10.1182/blood-2011-02-335216 |pmid=21566094}} External links{{Medical resources| DiseasesDB = | ICD10 = {{ICD10|C|86.2}} | ICD9 = | ICDO = {{ICDO|9717|3}} | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = }}{{Lymphoid malignancy}}{{DEFAULTSORT:Enteropathy-Associated T-Cell Lymphoma}} 2 : Lymphoma|Gastrointestinal tract disorders |
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