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Clonal T-Cell Receptor Gene Rearrangement

MedGen UID:
273185
•Concept ID:
C1516680
•
Cell or Molecular Dysfunction
Synonyms: Clonal TCR Gene Rearrangement; Monoclonal T-Cell Receptor Gene Rearrangement

Definition

Rearrangements involving DNA recombinase and T-cell receptor family genes that result in the expression of antigen-specific T-cell receptor proteins. This process occurs during the differentiation of T-lymphocytes, such that individual T-cell progenitors, and their descendents, express identical forms of the T-cell receptor complex. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVClonal T-Cell Receptor Gene Rearrangement

Professional guidelines

PubMed

Report of the Workshop on Nasal and Related Extranodal Angiocentric T/Natural Killer Cell Lymphomas. Definitions, differential diagnosis, and epidemiology.
Jaffe ES, Chan JK, Su IJ, Frizzera G, Mori S, Feller AC, Ho FC
Am J Surg Pathol 1996 Jan;20(1):103-11. doi: 10.1097/00000478-199601000-00012. PMID: 8540601

Recent clinical studies

Etiology

Cutaneous T-cell lymphomas with pathogenic somatic mutations and absence of detectable clonal T-cell receptor gene rearrangement: two case reports.
Rojansky R, Fernandez-Pol S, Wang E, Rieger KE, Novoa RA, Zehnder JL, Kunder CA, Kim YH, Khodadoust MS, Brown RA
Diagn Pathol 2020 Sep 28;15(1):122. doi: 10.1186/s13000-020-01022-x. PMID: 32988392Free PMC Article
Subcutaneous panniculitis-like T-cell lymphoma in the pediatric age group: a lymphoma of low malignant potential.
Huppmann AR, Xi L, Raffeld M, Pittaluga S, Jaffe ES
Pediatr Blood Cancer 2013 Jul;60(7):1165-70. Epub 2013 Feb 4 doi: 10.1002/pbc.24462. PMID: 23382035Free PMC Article
Clinicopathologic features of gastrointestinal tract involvement of anaplastic large cell lymphoma.
Sun J, Zhou JL, Chen J, Lu CH
Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2012 Jun;34(3):228-33. doi: 10.3881/j.issn.1000-503X.2012.03.007. PMID: 22776654
Primary follicular mucinosis: long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement.
Brown HA, Gibson LE, Pujol RM, Lust JA, Pittelkow MR
J Am Acad Dermatol 2002 Dec;47(6):856-62. doi: 10.1067/mjd.2002.124604. PMID: 12451369
Association of pure red cell aplasia with T large granular lymphocyte leukaemia.
Kwong YL, Wong KF
J Clin Pathol 1998 Sep;51(9):672-5. doi: 10.1136/jcp.51.9.672. PMID: 9930071Free PMC Article

Diagnosis

Cutaneous T-cell lymphomas with pathogenic somatic mutations and absence of detectable clonal T-cell receptor gene rearrangement: two case reports.
Rojansky R, Fernandez-Pol S, Wang E, Rieger KE, Novoa RA, Zehnder JL, Kunder CA, Kim YH, Khodadoust MS, Brown RA
Diagn Pathol 2020 Sep 28;15(1):122. doi: 10.1186/s13000-020-01022-x. PMID: 32988392Free PMC Article
Primary follicular mucinosis: long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement.
Brown HA, Gibson LE, Pujol RM, Lust JA, Pittelkow MR
J Am Acad Dermatol 2002 Dec;47(6):856-62. doi: 10.1067/mjd.2002.124604. PMID: 12451369
Giant cell granulomatous pulmonary and myocardial lesions in necrobiotic xanthogranuloma with paraproteinemia.
Winkelmann RK, Litzow MR, Umbert IJ, Lie JT
Mayo Clin Proc 1997 Nov;72(11):1028-33. doi: 10.4065/72.11.1028. PMID: 9374976
Routine diagnosis of large granular lymphocytic leukaemia by Southern blot and polymerase chain reaction analysis of clonal T cell receptor gene rearrangement.
Ryan DK, Alexander HD, Morris TC
Mol Pathol 1997 Apr;50(2):77-81. doi: 10.1136/mp.50.2.77. PMID: 9231154Free PMC Article
Partial T-cell receptor gene rearrangement. A source of pseudo-clonal populations in thymomas and other thymic tissues.
Pizer ES, McGrath SD, Hruban RH, Drachman DB, Bulkley GB, Zehnbauer BA
Am J Clin Pathol 1996 Mar;105(3):262-7. doi: 10.1093/ajcp/105.3.262. PMID: 8602605

Therapy

Comparison of Epstein-Barr virus-positive mucocutaneous ulcer associated with treated lymphoma or methotrexate in Japan.
Daroontum T, Kohno K, Eladl AE, Satou A, Sakakibara A, Matsukage S, Yakushiji N, Ya-In C, Nakamura S, Asano N, Kato S
Histopathology 2018 Jun;72(7):1115-1127. Epub 2018 Mar 7 doi: 10.1111/his.13464. PMID: 29314151
Mycosis fungoides involving an acrochordon: a case report.
McClain CM, Cole MB, Robbins JB, Kantrow SM
J Cutan Pathol 2012 Dec;39(12):1131-4. Epub 2012 Sep 21 doi: 10.1111/cup.12009. PMID: 22994930
Hepatosplenic gamma-delta T-cell lymphoma as a late-onset posttransplant lymphoproliferative disorder in renal transplant recipients.
Wu H, Wasik MA, Przybylski G, Finan J, Haynes B, Moore H, Leonard DG, Montone KT, Naji A, Nowell PC, Kamoun M, Tomaszewski JE, Salhany KE
Am J Clin Pathol 2000 Apr;113(4):487-96. doi: 10.1309/YTTC-F55W-K9CP-EPX5. PMID: 10761449
Demonstration of clonality, by X-linked DNA analysis, in chronic natural killer cell lymphocytosis and successful therapy with oral cyclophosphamide.
Tefferi A, Greipp PR, Leibson PJ, Thibodeau SN
Leukemia 1992 May;6(5):477-80. PMID: 1350651

Prognosis

Indolent, waxing and waning cutaneous presentation of HTLV-1-associated adult T-cell leukemia/lymphoma in an HIV-1-positive patient.
Richey JD, Chen BJ, Deng AC
J Cutan Pathol 2018 Feb;45(2):171-175. Epub 2017 Dec 10 doi: 10.1111/cup.13078. PMID: 29094771
Primary follicular mucinosis: long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement.
Brown HA, Gibson LE, Pujol RM, Lust JA, Pittelkow MR
J Am Acad Dermatol 2002 Dec;47(6):856-62. doi: 10.1067/mjd.2002.124604. PMID: 12451369
Association of pure red cell aplasia with T large granular lymphocyte leukaemia.
Kwong YL, Wong KF
J Clin Pathol 1998 Sep;51(9):672-5. doi: 10.1136/jcp.51.9.672. PMID: 9930071Free PMC Article
Report of the Workshop on Nasal and Related Extranodal Angiocentric T/Natural Killer Cell Lymphomas. Definitions, differential diagnosis, and epidemiology.
Jaffe ES, Chan JK, Su IJ, Frizzera G, Mori S, Feller AC, Ho FC
Am J Surg Pathol 1996 Jan;20(1):103-11. doi: 10.1097/00000478-199601000-00012. PMID: 8540601
Lymphoma with clonal T-cell receptor gene rearrangement in a 25-year survivor of Hodgkin's disease.
Li FP, Gimbrere K, Ritz J, Saito H, Longtine JA
Cancer 1987 Nov 1;60(9):2213-8. doi: 10.1002/1097-0142(19871101)60:9<2213::aid-cncr2820600918>3.0.co;2-j. PMID: 3501968

Clinical prediction guides

Comparison of Epstein-Barr virus-positive mucocutaneous ulcer associated with treated lymphoma or methotrexate in Japan.
Daroontum T, Kohno K, Eladl AE, Satou A, Sakakibara A, Matsukage S, Yakushiji N, Ya-In C, Nakamura S, Asano N, Kato S
Histopathology 2018 Jun;72(7):1115-1127. Epub 2018 Mar 7 doi: 10.1111/his.13464. PMID: 29314151
Relationship Between Pityriasis Lichenoides and Mycosis Fungoides: A Clinicopathological, Immunohistochemical, and Molecular Study.
Zaaroura H, Sahar D, Bick T, Bergman R
Am J Dermatopathol 2018 Jun;40(6):409-415. doi: 10.1097/DAD.0000000000001057. PMID: 29210716
Hypopigmented mycosis fungoides: frequent expression of a CD8+ T-cell phenotype.
El-Shabrawi-Caelen L, Cerroni L, Medeiros LJ, McCalmont TH
Am J Surg Pathol 2002 Apr;26(4):450-7. doi: 10.1097/00000478-200204000-00006. PMID: 11914622
Hepatosplenic gamma-delta T-cell lymphoma as a late-onset posttransplant lymphoproliferative disorder in renal transplant recipients.
Wu H, Wasik MA, Przybylski G, Finan J, Haynes B, Moore H, Leonard DG, Montone KT, Naji A, Nowell PC, Kamoun M, Tomaszewski JE, Salhany KE
Am J Clin Pathol 2000 Apr;113(4):487-96. doi: 10.1309/YTTC-F55W-K9CP-EPX5. PMID: 10761449
Frequent detection of Epstein-Barr virus-infected B cells in peripheral T-cell lymphomas.
Ho JW, Ho FC, Chan AC, Liang RH, Srivastava G
J Pathol 1998 May;185(1):79-85. doi: 10.1002/(SICI)1096-9896(199805)185:1<79::AID-PATH52>3.0.CO;2-3. PMID: 9713363

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