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Small Lymphocytic Lymphoma with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene

MedGen UID:
234793
•Concept ID:
C1336013
•
Neoplastic Process
Synonym: Pregerminal Center Small Lymphocytic Lymphoma

Definition

Small lymphocytic lymphoma 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  
  • CROGVSmall Lymphocytic Lymphoma 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
Prognostic relevance of the FAB morphological criteria in chronic lymphocytic leukemia: correlations with IgVH gene mutational status and other prognostic markers.
Schwarz J, Mikulenková D, Cermáková M, Polanská V, Michalová K, Marinov I, Campr V, Ransdorfová S, Marková J, Pavlistová L, Brezinová J, Sajdová J, Sponerová D, Volková Z, Benesová K, Cermák J, Vítek A, Cetkovský P
Neoplasma 2006;53(3):219-25. PMID: 16652191

Recent clinical studies

Etiology

Acalabrutinib-based regimens in frontline or relapsed/refractory higher-risk CLL: pooled analysis of 5 clinical trials.
Davids MS, Sharman JP, Ghia P, Woyach JA, Eyre TA, Jurczak W, Siddiqi T, Miranda P, Shahkarami M, Butturini A, Emeribe U, Byrd JC
Blood Adv 2024 Jul 9;8(13):3345-3359. doi: 10.1182/bloodadvances.2023011307. PMID: 38640349Free PMC Article
STEREOTYPED CASES IN UKRAINIAN COHORT OF CHRONIC LYMPHOCYTIC LEUKEMIA PATIENTS DEPENDING ON THE IONIZING RADIATION EXPOSURE.
Bilous NI, Abramenko IV, Chumak AA, Dyagil IS, Martina ZV
Probl Radiac Med Radiobiol 2022 Dec;27:307-323. doi: 10.33145/2304-8336-2022-27-307-323. PMID: 36582097
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
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
Genetics of chronic lymphocytic leukemia: genomic aberrations and V(H) gene mutation status in pathogenesis and clinical course.
Stilgenbauer S, Bullinger L, Lichter P, Döhner H; German CLL Study Group (GCLLSG). Chronic lymphocytic leukemia
Leukemia 2002 Jun;16(6):993-1007. doi: 10.1038/sj.leu.2402537. PMID: 12040431

Diagnosis

Clinical Outcomes in Patients with Multi-Hit TP53 Chronic Lymphocytic Leukemia Treated with Ibrutinib.
Brieghel C, Aarup K, Torp MH, Andersen MA, Yde CW, Tian X, Wiestner A, Ahn IE, Niemann CU
Clin Cancer Res 2021 Aug 15;27(16):4531-4538. Epub 2021 May 7 doi: 10.1158/1078-0432.CCR-20-4890. PMID: 33963002Free 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
Cryptochrome-1 Gene Expression is a Reliable Prognostic Indicator in Egyptian Patients with Chronic Lymphocytic Leukemia: A Prospective Cohort Study.
Habashy DM, Eissa DS, Aboelez MM
Turk J Haematol 2018 Aug 3;35(3):168-174. Epub 2017 Sep 8 doi: 10.4274/tjh.2017.0169. PMID: 28884705Free PMC Article
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
Genetics of chronic lymphocytic leukemia: genomic aberrations and V(H) gene mutation status in pathogenesis and clinical course.
Stilgenbauer S, Bullinger L, Lichter P, Döhner H; German CLL Study Group (GCLLSG). Chronic lymphocytic leukemia
Leukemia 2002 Jun;16(6):993-1007. doi: 10.1038/sj.leu.2402537. PMID: 12040431

Therapy

STEREOTYPED CASES IN UKRAINIAN COHORT OF CHRONIC LYMPHOCYTIC LEUKEMIA PATIENTS DEPENDING ON THE IONIZING RADIATION EXPOSURE.
Bilous NI, Abramenko IV, Chumak AA, Dyagil IS, Martina ZV
Probl Radiac Med Radiobiol 2022 Dec;27:307-323. doi: 10.33145/2304-8336-2022-27-307-323. PMID: 36582097
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
Clinical Outcomes in Patients with Multi-Hit TP53 Chronic Lymphocytic Leukemia Treated with Ibrutinib.
Brieghel C, Aarup K, Torp MH, Andersen MA, Yde CW, Tian X, Wiestner A, Ahn IE, Niemann CU
Clin Cancer Res 2021 Aug 15;27(16):4531-4538. Epub 2021 May 7 doi: 10.1158/1078-0432.CCR-20-4890. PMID: 33963002Free 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

Prognosis

Acalabrutinib-based regimens in frontline or relapsed/refractory higher-risk CLL: pooled analysis of 5 clinical trials.
Davids MS, Sharman JP, Ghia P, Woyach JA, Eyre TA, Jurczak W, Siddiqi T, Miranda P, Shahkarami M, Butturini A, Emeribe U, Byrd JC
Blood Adv 2024 Jul 9;8(13):3345-3359. doi: 10.1182/bloodadvances.2023011307. PMID: 38640349Free PMC Article
Clinical Outcomes in Patients with Multi-Hit TP53 Chronic Lymphocytic Leukemia Treated with Ibrutinib.
Brieghel C, Aarup K, Torp MH, Andersen MA, Yde CW, Tian X, Wiestner A, Ahn IE, Niemann CU
Clin Cancer Res 2021 Aug 15;27(16):4531-4538. Epub 2021 May 7 doi: 10.1158/1078-0432.CCR-20-4890. PMID: 33963002Free PMC Article
NDRG2 mRNA levels and miR-28-5p and miR-650 activity in chronic lymphocytic leukemia.
Yang YQ, Tian T, Zhu HY, Liang JH, Wu W, Wu JZ, Xia Y, Wang L, Fan L, Li JY, Xu W
BMC Cancer 2018 Oct 22;18(1):1009. doi: 10.1186/s12885-018-4915-3. PMID: 30348117Free PMC Article
Immunogenetics of chronic lymphocytic leukemia.
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
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

Clinical prediction guides

Acalabrutinib, venetoclax, and obinutuzumab in relapsed/refractory CLL: final efficacy and ctDNA analysis of the CLL2-BAAG trial.
Fürstenau M, Giza A, Weiss J, Kleinert F, Robrecht S, Franzen F, Stumpf J, Langerbeins P, Al-Sawaf O, Simon F, Fink AM, Schneider C, Tausch E, Schetelig J, Dreger P, Böttcher S, Fischer K, Kreuzer KA, Ritgen M, Schilhabel A, Brüggemann M, Stilgenbauer S, Eichhorst B, Hallek M, Cramer P
Blood 2024 Jul 18;144(3):272-282. doi: 10.1182/blood.2023022730. PMID: 38620072
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
Comprehensive assessment of genetic and molecular features predicting outcome in patients with chronic lymphocytic leukemia: results from the US Intergroup Phase III Trial E2997.
Grever MR, Lucas DM, Dewald GW, Neuberg DS, Reed JC, Kitada S, Flinn IW, Tallman MS, Appelbaum FR, Larson RA, Paietta E, Jelinek DF, Gribben JG, Byrd JC
J Clin Oncol 2007 Mar 1;25(7):799-804. Epub 2007 Feb 5 doi: 10.1200/JCO.2006.08.3089. PMID: 17283363
The origin of B-cell chronic lymphocytic leukemia.
Ghia P, Caligaris-Cappio F
Semin Oncol 2006 Apr;33(2):150-6. doi: 10.1053/j.seminoncol.2006.01.009. PMID: 16616061
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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