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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

Bruton tyrosine kinase inhibitors for the frontline treatment of chronic lymphocytic leukemia.
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
Evans syndrome secondary to chronic lymphocytic leukaemia: presentation, treatment, and outcome.
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
Clinical utility of molecular and flow cytometric markers in chronic lymphocytic leukaemia.
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

Orelabrutinib in relapsed or refractory chronic lymphocytic leukemia/small lymphocytic lymphoma patients: Multi-center, single-arm, open-label, phase 2 study.
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
Targeting Bruton's Tyrosine Kinase in CLL.
Ahn IE, Brown JR
Front Immunol 2021;12:687458. Epub 2021 Jun 23 doi: 10.3389/fimmu.2021.687458. PMID: 34248972Free PMC Article
Bruton tyrosine kinase inhibitors for the frontline treatment of chronic lymphocytic leukemia.
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
Distinct immunoglobulin heavy chain variable region gene repertoire and lower frequency of del(11q) in Taiwanese patients with chronic lymphocytic leukaemia.
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
Genotypic prognostic markers.
Stilgenbauer S, Döhner H
Curr Top Microbiol Immunol 2005;294:147-64. doi: 10.1007/3-540-29933-5_9. PMID: 16323431

Diagnosis

Bruton tyrosine kinase inhibitors for the frontline treatment of chronic lymphocytic leukemia.
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
Distinct immunoglobulin heavy chain variable region gene repertoire and lower frequency of del(11q) in Taiwanese patients with chronic lymphocytic leukaemia.
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
Immunoglobulin heavy chain variable region gene repertoire and B-cell receptor stereotypes in Indian patients with chronic lymphocytic leukemia.
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
Molecular basis of chronic lymphocytic leukemia diagnosis and prognosis.
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
Molecular pathogenesis of chronic lymphocytic leukemia.
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

Orelabrutinib in relapsed or refractory chronic lymphocytic leukemia/small lymphocytic lymphoma patients: Multi-center, single-arm, open-label, phase 2 study.
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
Long-term outcomes for ibrutinib-rituximab and chemoimmunotherapy in CLL: updated results of the E1912 trial.
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
Targeting Bruton's Tyrosine Kinase in CLL.
Ahn IE, Brown JR
Front Immunol 2021;12:687458. Epub 2021 Jun 23 doi: 10.3389/fimmu.2021.687458. PMID: 34248972Free PMC Article
Bruton tyrosine kinase inhibitors for the frontline treatment of chronic lymphocytic leukemia.
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
Genotypic prognostic markers.
Stilgenbauer S, Döhner H
Curr Top Microbiol Immunol 2005;294:147-64. doi: 10.1007/3-540-29933-5_9. PMID: 16323431

Prognosis

Orelabrutinib in relapsed or refractory chronic lymphocytic leukemia/small lymphocytic lymphoma patients: Multi-center, single-arm, open-label, phase 2 study.
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
Immunoglobulin heavy chain variable region gene repertoire and B-cell receptor stereotypes in Indian patients with chronic lymphocytic leukemia.
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
Molecular basis of chronic lymphocytic leukemia diagnosis and prognosis.
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
MicroRNAs and B cell receptor signaling in chronic lymphocytic leukemia.
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
Genotypic prognostic markers.
Stilgenbauer S, Döhner H
Curr Top Microbiol Immunol 2005;294:147-64. doi: 10.1007/3-540-29933-5_9. PMID: 16323431

Clinical prediction guides

Long-term outcomes for ibrutinib-rituximab and chemoimmunotherapy in CLL: updated results of the E1912 trial.
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
The Biology of Chronic Lymphocytic Leukemia: Diagnostic and Prognostic Implications.
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
Extracellular vesicles and their associated miRNAs as potential prognostic biomarkers in chronic lymphocytic leukemia.
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
Immunoglobulin heavy chain variable region gene repertoire and B-cell receptor stereotypes in Indian patients with chronic lymphocytic leukemia.
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
Genotypic prognostic markers.
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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