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Chronic Lymphocytic Leukemia with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene

MedGen UID:
234147
Concept ID:
C1333041
Neoplastic Process
Synonym: Pregerminal Center Chronic Lymphocytic Leukemia

Definition

Chronic lymphocytic leukemia characterized by the absence of somatic hypermutation within the immunoglobulin heavy chain gene variable region of neoplastic clones. Patients have a significantly worse prognosis as compared to those with mutated immunoglobulin heavy chain gene variable region rearrangements. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic Lymphocytic Leukemia with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene

Professional guidelines

PubMed

Banerji V, Aw A, Robinson S, Doucette S, Christofides A, Sehn LH
Curr Oncol 2020 Dec;27(6):e645-e655. Epub 2020 Dec 1 doi: 10.3747/co.27.6795. PMID: 33380880Free PMC Article
Carli G, Visco C, Falisi E, Perbellini O, Novella E, Giaretta I, Ferrarini I, Sandini A, Alghisi A, Ambrosetti A, Rodeghiero F
Ann Hematol 2016 May;95(6):863-70. Epub 2016 Mar 22 doi: 10.1007/s00277-016-2642-x. PMID: 27001309
Sulda ML, Kuss BJ, Hall RK, Bailey S, Macardle PJ
Intern Med J 2012 Feb;42(2):137-46. Epub 2010 Jun 16 doi: 10.1111/j.1445-5994.2010.02294.x. PMID: 20561095

Recent clinical studies

Etiology

Xu W, Zhou K, Wang T, Yang S, Liu L, Hu Y, Zhang W, Ding K, Zhou J, Gao S, Xu B, Zhu Z, Liu T, Zhang H, Hu J, Ji C, Wang S, Xia Z, Wang X, Li Y, Song Y, Ma S, Tang X, Zhang B, Li J
Am J Hematol 2023 Apr;98(4):571-579. Epub 2023 Jan 22 doi: 10.1002/ajh.26826. PMID: 36683422
Ahn IE, Brown JR
Front Immunol 2021;12:687458. Epub 2021 Jun 23 doi: 10.3389/fimmu.2021.687458. PMID: 34248972Free PMC Article
Banerji V, Aw A, Robinson S, Doucette S, Christofides A, Sehn LH
Curr Oncol 2020 Dec;27(6):e645-e655. Epub 2020 Dec 1 doi: 10.3747/co.27.6795. PMID: 33380880Free PMC Article
Huang YJ, Kuo MC, Chang H, Wang PN, Wu JH, Huang YM, Ma MC, Tang TC, Kuo CY, Shih LY
Br J Haematol 2019 Oct;187(1):82-92. Epub 2019 Jun 23 doi: 10.1111/bjh.16051. PMID: 31230372Free PMC Article
Stilgenbauer S, Döhner H
Curr Top Microbiol Immunol 2005;294:147-64. doi: 10.1007/3-540-29933-5_9. PMID: 16323431

Diagnosis

Moia R, Patriarca A, Schipani M, Gaidano G
Cancer J 2021 Jul-Aug 01;27(4):266-274. doi: 10.1097/PPO.0000000000000534. PMID: 34398553
Banerji V, Aw A, Robinson S, Doucette S, Christofides A, Sehn LH
Curr Oncol 2020 Dec;27(6):e645-e655. Epub 2020 Dec 1 doi: 10.3747/co.27.6795. PMID: 33380880Free PMC Article
Huang YJ, Kuo MC, Chang H, Wang PN, Wu JH, Huang YM, Ma MC, Tang TC, Kuo CY, Shih LY
Br J Haematol 2019 Oct;187(1):82-92. Epub 2019 Jun 23 doi: 10.1111/bjh.16051. PMID: 31230372Free PMC Article
Shahjahani M, Mohammadiasl J, Noroozi F, Seghatoleslami M, Shahrabi S, Saba F, Saki N
Cell Oncol (Dordr) 2015 Apr;38(2):93-109. Epub 2015 Jan 7 doi: 10.1007/s13402-014-0215-3. PMID: 25563586
Gaidano G, Foà R, Dalla-Favera R
J Clin Invest 2012 Oct;122(10):3432-8. Epub 2012 Oct 1 doi: 10.1172/JCI64101. PMID: 23023714Free PMC Article

Therapy

Xu W, Zhou K, Wang T, Yang S, Liu L, Hu Y, Zhang W, Ding K, Zhou J, Gao S, Xu B, Zhu Z, Liu T, Zhang H, Hu J, Ji C, Wang S, Xia Z, Wang X, Li Y, Song Y, Ma S, Tang X, Zhang B, Li J
Am J Hematol 2023 Apr;98(4):571-579. Epub 2023 Jan 22 doi: 10.1002/ajh.26826. PMID: 36683422
Shanafelt TD, Wang XV, Hanson CA, Paietta EM, O'Brien S, Barrientos J, Jelinek DF, Braggio E, Leis JF, Zhang CC, Coutre SE, Barr PM, Cashen AF, Mato AR, Singh AK, Mullane MP, Little RF, Erba H, Stone RM, Litzow M, Tallman M, Kay NE
Blood 2022 Jul 14;140(2):112-120. doi: 10.1182/blood.2021014960. PMID: 35427411Free PMC Article
Ahn IE, Brown JR
Front Immunol 2021;12:687458. Epub 2021 Jun 23 doi: 10.3389/fimmu.2021.687458. PMID: 34248972Free PMC Article
Banerji V, Aw A, Robinson S, Doucette S, Christofides A, Sehn LH
Curr Oncol 2020 Dec;27(6):e645-e655. Epub 2020 Dec 1 doi: 10.3747/co.27.6795. PMID: 33380880Free PMC Article
Stilgenbauer S, Döhner H
Curr Top Microbiol Immunol 2005;294:147-64. doi: 10.1007/3-540-29933-5_9. PMID: 16323431

Prognosis

Xu W, Zhou K, Wang T, Yang S, Liu L, Hu Y, Zhang W, Ding K, Zhou J, Gao S, Xu B, Zhu Z, Liu T, Zhang H, Hu J, Ji C, Wang S, Xia Z, Wang X, Li Y, Song Y, Ma S, Tang X, Zhang B, Li J
Am J Hematol 2023 Apr;98(4):571-579. Epub 2023 Jan 22 doi: 10.1002/ajh.26826. PMID: 36683422
Patkar N, Rabade N, Kadam PA, Mishra F, Muranjan A, Tembhare P, Chaudhary S, Joshi S, Jain H, Dangi U, Bagal B, Khattry N, Menon H, Gujral S, Sengar M, Subramanian PG
Indian J Pathol Microbiol 2017 Jan-Mar;60(1):38-42. doi: 10.4103/0377-4929.200051. PMID: 28195089
Shahjahani M, Mohammadiasl J, Noroozi F, Seghatoleslami M, Shahrabi S, Saba F, Saki N
Cell Oncol (Dordr) 2015 Apr;38(2):93-109. Epub 2015 Jan 7 doi: 10.1007/s13402-014-0215-3. PMID: 25563586
Mraz M, Kipps TJ
Leuk Lymphoma 2013 Aug;54(8):1836-9. Epub 2013 Jun 12 doi: 10.3109/10428194.2013.796055. PMID: 23597135Free PMC Article
Gaidano G, Foà R, Dalla-Favera R
J Clin Invest 2012 Oct;122(10):3432-8. Epub 2012 Oct 1 doi: 10.1172/JCI64101. PMID: 23023714Free PMC Article

Clinical prediction guides

Shanafelt TD, Wang XV, Hanson CA, Paietta EM, O'Brien S, Barrientos J, Jelinek DF, Braggio E, Leis JF, Zhang CC, Coutre SE, Barr PM, Cashen AF, Mato AR, Singh AK, Mullane MP, Little RF, Erba H, Stone RM, Litzow M, Tallman M, Kay NE
Blood 2022 Jul 14;140(2):112-120. doi: 10.1182/blood.2021014960. PMID: 35427411Free PMC Article
Moia R, Patriarca A, Schipani M, Gaidano G
Cancer J 2021 Jul-Aug 01;27(4):266-274. doi: 10.1097/PPO.0000000000000534. PMID: 34398553
Aguilar-Hernandez MM, Rincon Camacho JC, Galicia Garcia G
Curr Oncol Rep 2021 Apr 14;23(6):66. doi: 10.1007/s11912-021-01058-2. PMID: 33855607
Rani L, Mathur N, Gogia A, Vishnubhatla S, Kumar L, Sharma A, Dube D, Kaur P, Gupta R
Leuk Lymphoma 2016 Oct;57(10):2389-400. Epub 2016 Mar 4 doi: 10.3109/10428194.2016.1153086. PMID: 26942309
Stilgenbauer S, Döhner H
Curr Top Microbiol Immunol 2005;294:147-64. doi: 10.1007/3-540-29933-5_9. PMID: 16323431

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