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NK-cell enteropathy

MedGen UID:
1379183
•Concept ID:
C4509932
•
Neoplastic Process
Synonym: Natural killer cell enteropathy
SNOMED CT: Natural killer cell enteropathy (723496007); NK-cell enteropathy (723496007); NK (natural killer) cell enteropathy (723496007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016996
Orphanet: ORPHA263665

Definition

A benign natural killer (NK) cell lymphoproliferative disease with characteristics of minor abdominal symptoms (abdominal pain, diverticulosis, constipation and reflux) due to NK cell-derived lesions in the mucosal layer of the gastrointestinal tract and often mistaken for NK or T-cell lymphoma. [from SNOMEDCT_US]

Professional guidelines

PubMed

The double-edged sword of probiotic supplementation on gut microbiota structure in Helicobacter pylori management.
Nabavi-Rad A, Sadeghi A, Asadzadeh Aghdaei H, Yadegar A, Smith SM, Zali MR
Gut Microbes 2022 Jan-Dec;14(1):2108655. doi: 10.1080/19490976.2022.2108655. PMID: 35951774Free PMC Article
Targeted suppression of human IBD-associated gut microbiota commensals by phage consortia for treatment of intestinal inflammation.
Federici S, Kredo-Russo S, Valdés-Mas R, Kviatcovsky D, Weinstock E, Matiuhin Y, Silberberg Y, Atarashi K, Furuichi M, Oka A, Liu B, Fibelman M, Weiner IN, Khabra E, Cullin N, Ben-Yishai N, Inbar D, Ben-David H, Nicenboim J, Kowalsman N, Lieb W, Kario E, Cohen T, Geffen YF, Zelcbuch L, Cohen A, Rappo U, Gahali-Sass I, Golembo M, Lev V, Dori-Bachash M, Shapiro H, Moresi C, Cuevas-Sierra A, Mohapatra G, Kern L, Zheng D, Nobs SP, Suez J, Stettner N, Harmelin A, Zak N, Puttagunta S, Bassan M, Honda K, Sokol H, Bang C, Franke A, Schramm C, Maharshak N, Sartor RB, Sorek R, Elinav E
Cell 2022 Aug 4;185(16):2879-2898.e24. doi: 10.1016/j.cell.2022.07.003. PMID: 35931020
Cocultures of human colorectal tumor spheroids with immune cells reveal the therapeutic potential of MICA/B and NKG2A targeting for cancer treatment.
Courau T, Bonnereau J, Chicoteau J, Bottois H, Remark R, Assante Miranda L, Toubert A, Blery M, Aparicio T, Allez M, Le Bourhis L
J Immunother Cancer 2019 Mar 14;7(1):74. doi: 10.1186/s40425-019-0553-9. PMID: 30871626Free PMC Article

Recent clinical studies

Etiology

Diagnostic approach to T- and NK-cell lymphoproliferative disorders in the gastrointestinal tract.
Susan SH, Ng SB, Wang S, Tan SY
Semin Diagn Pathol 2021 Jul;38(4):21-30. Epub 2021 Mar 31 doi: 10.1053/j.semdp.2021.03.004. PMID: 34016481
T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update.
van Vliet C, Spagnolo DV
Pathology 2020 Jan;52(1):128-141. Epub 2019 Nov 11 doi: 10.1016/j.pathol.2019.10.001. PMID: 31727264
Gastrointestinal T- and NK-cell lymphomas and indolent lymphoproliferative disorders.
Soderquist CR, Bhagat G
Semin Diagn Pathol 2020 Jan;37(1):11-23. Epub 2019 Aug 28 doi: 10.1053/j.semdp.2019.08.001. PMID: 31522873
Early lymphoid lesions: conceptual, diagnostic and clinical challenges.
Ganapathi KA, Pittaluga S, Odejide OO, Freedman AS, Jaffe ES
Haematologica 2014 Sep;99(9):1421-32. doi: 10.3324/haematol.2014.107938. PMID: 25176983Free PMC Article
NK-cell enteropathy: a benign NK-cell lymphoproliferative disease mimicking intestinal lymphoma: clinicopathologic features and follow-up in a unique case series.
Mansoor A, Pittaluga S, Beck PL, Wilson WH, Ferry JA, Jaffe ES
Blood 2011 Feb 3;117(5):1447-52. Epub 2010 Oct 21 doi: 10.1182/blood-2010-08-302737. PMID: 20966166Free PMC Article

Diagnosis

Diagnostic approach to T- and NK-cell lymphoproliferative disorders in the gastrointestinal tract.
Susan SH, Ng SB, Wang S, Tan SY
Semin Diagn Pathol 2021 Jul;38(4):21-30. Epub 2021 Mar 31 doi: 10.1053/j.semdp.2021.03.004. PMID: 34016481
Lymph node involvement by enteropathy-like indolent NK-cell proliferation.
Dargent JL, Tinton N, Trimech M, de Leval L
Virchows Arch 2021 Jun;478(6):1197-1202. Epub 2020 Jul 21 doi: 10.1007/s00428-020-02892-8. PMID: 32696224Free PMC Article
T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update.
van Vliet C, Spagnolo DV
Pathology 2020 Jan;52(1):128-141. Epub 2019 Nov 11 doi: 10.1016/j.pathol.2019.10.001. PMID: 31727264
Gastrointestinal T- and NK-cell lymphomas and indolent lymphoproliferative disorders.
Soderquist CR, Bhagat G
Semin Diagn Pathol 2020 Jan;37(1):11-23. Epub 2019 Aug 28 doi: 10.1053/j.semdp.2019.08.001. PMID: 31522873
NK-Cell Enteropathy and Similar Indolent Lymphoproliferative Disorders: A Case Series With Literature Review.
Xia D, Morgan EA, Berger D, Pinkus GS, Ferry JA, Zukerberg LR
Am J Clin Pathol 2019 Jan 1;151(1):75-85. doi: 10.1093/ajcp/aqy108. PMID: 30212873

Therapy

Early lymphoid lesions: conceptual, diagnostic and clinical challenges.
Ganapathi KA, Pittaluga S, Odejide OO, Freedman AS, Jaffe ES
Haematologica 2014 Sep;99(9):1421-32. doi: 10.3324/haematol.2014.107938. PMID: 25176983Free PMC Article
NK-cell enteropathy: a benign NK-cell lymphoproliferative disease mimicking intestinal lymphoma: clinicopathologic features and follow-up in a unique case series.
Mansoor A, Pittaluga S, Beck PL, Wilson WH, Ferry JA, Jaffe ES
Blood 2011 Feb 3;117(5):1447-52. Epub 2010 Oct 21 doi: 10.1182/blood-2010-08-302737. PMID: 20966166Free PMC Article

Prognosis

Diagnostic approach to T- and NK-cell lymphoproliferative disorders in the gastrointestinal tract.
Susan SH, Ng SB, Wang S, Tan SY
Semin Diagn Pathol 2021 Jul;38(4):21-30. Epub 2021 Mar 31 doi: 10.1053/j.semdp.2021.03.004. PMID: 34016481
T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update.
van Vliet C, Spagnolo DV
Pathology 2020 Jan;52(1):128-141. Epub 2019 Nov 11 doi: 10.1016/j.pathol.2019.10.001. PMID: 31727264
An Enteropathy-like Indolent NK-Cell Proliferation Presenting in the Female Genital Tract.
Krishnan R, Ring K, Williams E, Portell C, Jaffe ES, Gru AA
Am J Surg Pathol 2020 Apr;44(4):561-565. doi: 10.1097/PAS.0000000000001387. PMID: 31609783Free PMC Article
NK cell enteropathy: a case report with 10 years of indolent clinical behaviour.
Isom JA, Arroyo MR, Reddy D, Joshi-Guske P, Al-Quran SZ, Li Y, Allan RW
Histopathology 2018 Aug;73(2):345-350. Epub 2018 May 21 doi: 10.1111/his.13502. PMID: 29474745
Early lymphoid lesions: conceptual, diagnostic and clinical challenges.
Ganapathi KA, Pittaluga S, Odejide OO, Freedman AS, Jaffe ES
Haematologica 2014 Sep;99(9):1421-32. doi: 10.3324/haematol.2014.107938. PMID: 25176983Free PMC Article

Clinical prediction guides

A case of excisionally remitted indolent NK-cell enteropathy in the oral cavity and a mini-review.
Li X, Li Z, Zhou X, Zheng Y, Zhang Y, Xie J
J Cutan Pathol 2024 Jul;51(7):518-524. Epub 2024 Apr 20 doi: 10.1111/cup.14626. PMID: 38642003
Lymphomatoid gastropathy/NK-cell enteropathy involving the stomach and intestine.
Nakajima M, Shimoda M, Takeuchi K, Dobashi A, Kanai T, Kanai Y, Iwao Y
J Clin Exp Hematop 2022 Jun 28;62(2):114-118. Epub 2022 Apr 27 doi: 10.3960/jslrt.21032. PMID: 35474034Free PMC Article
Natural Killer-cell Enteropathy of the Stomach in an Elderly Woman: A Case Report.
Koo YY, Lee J, Kim B, Jeong SJ, Oh EH, Park YE, Park J, Kim TO
Korean J Gastroenterol 2021 Dec 25;78(6):349-352. doi: 10.4166/kjg.2021.116. PMID: 34955512

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