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B-cell chronic lymphocytic leukemia(CLL)

MedGen UID:
44120
Concept ID:
C0023434
Neoplastic Process
Synonyms: Chronic lymphatic leukemia; Chronic lymphocytic leukemia; Familial Chronic Lymphocytic Leukemia; Leukemia, B-cell, chronic
SNOMED CT: CLL - Chronic lymphocytic leukemia (92814006); Chronic lymphoid leukemia (92814006); Chronic lymphocytic leukemia, B-cell type (51092000); B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (51092000); Malignant lymphoma lymphocytic well differentiated diffuse (51092000); B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (92814006); B-cell chronic lymphocytic leukemia (92814006); B-cell CLL/SLL (chronic lymphocytic leukemia/small lymphocytic lymphoma) (92814006); Chronic lymphoid leukemia, disease (92814006); Chronic lymphocytic leukemia (51092000); Chronic lymphoid leukemia (51092000); Chronic lymphatic leukemia (51092000)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0005550
Monarch Initiative: MONDO:0004948
OMIM®: 151400
Orphanet: ORPHA67038

Definition

Chronic lymphocytic leukemia (CLL) is a common neoplasia of B lymphocytes in which these cells progressively accumulate in the bone marrow, blood, and lymphoid tissues. The clinical evolution of the disorder is heterogeneous, with some patients having indolent disease and others having aggressive disease and short survival (summary by Quesada et al., 2012). Genetic Heterogeneity of Susceptibility to Chronic Lymphocytic Leukemia Susceptibility loci have been mapped to chromosomes 11p11 (CLLS1; 609630) and 13q14 (CLLS2; 109543) by genomewide linkage analysis and translocation studies, respectively. Susceptibility mapping to chromosome 9q34 (CLLS3; 612557) is associated with downregulation of the DAPK1 gene (600831). Genomewide association studies have identified susceptibility loci on chromosomes 6p25.3 (CLLS4; 612558) and 11q24.1 (CLLS5; 612559). [from OMIM]

Clinical features

From HPO
B-cell chronic lymphocytic leukemia
MedGen UID:
44120
Concept ID:
C0023434
Neoplastic Process
Chronic lymphocytic leukemia (CLL) is a common neoplasia of B lymphocytes in which these cells progressively accumulate in the bone marrow, blood, and lymphoid tissues. The clinical evolution of the disorder is heterogeneous, with some patients having indolent disease and others having aggressive disease and short survival (summary by Quesada et al., 2012). Genetic Heterogeneity of Susceptibility to Chronic Lymphocytic Leukemia Susceptibility loci have been mapped to chromosomes 11p11 (CLLS1; 609630) and 13q14 (CLLS2; 109543) by genomewide linkage analysis and translocation studies, respectively. Susceptibility mapping to chromosome 9q34 (CLLS3; 612557) is associated with downregulation of the DAPK1 gene (600831). Genomewide association studies have identified susceptibility loci on chromosomes 6p25.3 (CLLS4; 612558) and 11q24.1 (CLLS5; 612559).
Cellular immunodeficiency
MedGen UID:
344444
Concept ID:
C1855204
Finding
An immunodeficiency characterized by defective cell-mediated immunity or humoral immunity.

Conditions with this feature

B-cell chronic lymphocytic leukemia
MedGen UID:
44120
Concept ID:
C0023434
Neoplastic Process
Chronic lymphocytic leukemia (CLL) is a common neoplasia of B lymphocytes in which these cells progressively accumulate in the bone marrow, blood, and lymphoid tissues. The clinical evolution of the disorder is heterogeneous, with some patients having indolent disease and others having aggressive disease and short survival (summary by Quesada et al., 2012). Genetic Heterogeneity of Susceptibility to Chronic Lymphocytic Leukemia Susceptibility loci have been mapped to chromosomes 11p11 (CLLS1; 609630) and 13q14 (CLLS2; 109543) by genomewide linkage analysis and translocation studies, respectively. Susceptibility mapping to chromosome 9q34 (CLLS3; 612557) is associated with downregulation of the DAPK1 gene (600831). Genomewide association studies have identified susceptibility loci on chromosomes 6p25.3 (CLLS4; 612558) and 11q24.1 (CLLS5; 612559).
Leukemia, chronic lymphocytic, susceptibility to, 2
MedGen UID:
358385
Concept ID:
C1868683
Finding
Immunodeficiency 36
MedGen UID:
863371
Concept ID:
C4014934
Disease or Syndrome
Immunodeficiency-36 with lymphoproliferation (IMD36) is an autosomal dominant primary immunodeficiency with a highly heterogeneous clinical phenotype, characterized primarily by recurrent respiratory tract infections, lymphoproliferation, and antibody deficiency. Other features include growth retardation, mild neurodevelopmental delay, and autoimmunity. The major complication is development of B-cell lymphoma (Elkaim et al., 2016).

Professional guidelines

PubMed

Bhowmick M, Auckbarallee F, Edgar P, Ray A, Dasgupta S
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Coles AJ
Semin Neurol 2013 Feb;33(1):66-73. Epub 2013 May 25 doi: 10.1055/s-0033-1343797. PMID: 23709214
Lobetti-Bodoni C, Bertoni F, Stussi G, Cavalli F, Zucca E
Eur J Intern Med 2013 Jul;24(5):401-10. Epub 2013 Apr 11 doi: 10.1016/j.ejim.2013.03.009. PMID: 23583413

Recent clinical studies

Etiology

Hampel PJ, Parikh SA
Blood Cancer J 2022 Nov 29;12(11):161. doi: 10.1038/s41408-022-00756-9. PMID: 36446777Free PMC Article
Eichhorst B, Robak T, Montserrat E, Ghia P, Niemann CU, Kater AP, Gregor M, Cymbalista F, Buske C, Hillmen P, Hallek M, Mey U; ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2021 Jan;32(1):23-33. Epub 2020 Oct 19 doi: 10.1016/j.annonc.2020.09.019. PMID: 33091559
Sharma S, Rai KR
Cancer 2019 May 1;125(9):1432-1440. Epub 2019 Feb 26 doi: 10.1002/cncr.31931. PMID: 30807655
Hallek M, Shanafelt TD, Eichhorst B
Lancet 2018 Apr 14;391(10129):1524-1537. Epub 2018 Feb 21 doi: 10.1016/S0140-6736(18)30422-7. PMID: 29477250
Scarfò L, Ferreri AJ, Ghia P
Crit Rev Oncol Hematol 2016 Aug;104:169-82. Epub 2016 Jun 16 doi: 10.1016/j.critrevonc.2016.06.003. PMID: 27370174

Diagnosis

Chiorazzi N, Chen SS, Rai KR
Cold Spring Harb Perspect Med 2021 Feb 1;11(2) doi: 10.1101/cshperspect.a035220. PMID: 32229611Free PMC Article
Strati P, Jain N, O'Brien S
Mayo Clin Proc 2018 May;93(5):651-664. doi: 10.1016/j.mayocp.2018.03.002. PMID: 29728204
Scarfò L, Ferreri AJ, Ghia P
Crit Rev Oncol Hematol 2016 Aug;104:169-82. Epub 2016 Jun 16 doi: 10.1016/j.critrevonc.2016.06.003. PMID: 27370174
Woyach JA, Johnson AJ, Byrd JC
Semin Oncol 2016 Apr;43(2):199-200. Epub 2016 Feb 6 doi: 10.1053/j.seminoncol.2016.02.001. PMID: 27040696
Hallek M
Oncol Res Treat 2016;39(1-2):8. Epub 2016 Jan 22 doi: 10.1159/000443821. PMID: 26889878

Therapy

Ryan CE, Davids MS, Hermann R, Shahkarami M, Biondo J, Abhyankar S, Alhasani H, Sharman JP, Mato AR, Roeker LE
Future Oncol 2022 Oct;18(33):3689-3699. Epub 2022 Sep 14 doi: 10.2217/fon-2022-0456. PMID: 36102212
Hallek M, Al-Sawaf O
Am J Hematol 2021 Dec 1;96(12):1679-1705. doi: 10.1002/ajh.26367. PMID: 34625994
Hillmen P, Brown JR, Eichhorst BF, Lamanna N, O'Brien SM, Qiu L, Salmi T, Hilger J, Wu K, Cohen A, Huang J, Tam CS
Future Oncol 2020 Apr;16(10):517-523. Epub 2020 Mar 24 doi: 10.2217/fon-2019-0844. PMID: 32207333
Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Döhner H, Hillmen P, Keating M, Montserrat E, Chiorazzi N, Stilgenbauer S, Rai KR, Byrd JC, Eichhorst B, O'Brien S, Robak T, Seymour JF, Kipps TJ
Blood 2018 Jun 21;131(25):2745-2760. Epub 2018 Mar 14 doi: 10.1182/blood-2017-09-806398. PMID: 29540348
Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Döhner H, Hillmen P, Keating MJ, Montserrat E, Rai KR, Kipps TJ; International Workshop on Chronic Lymphocytic Leukemia
Blood 2008 Jun 15;111(12):5446-56. Epub 2008 Jan 23 doi: 10.1182/blood-2007-06-093906. PMID: 18216293Free PMC Article

Prognosis

Byrd JC, Hillmen P, Ghia P, Kater AP, Chanan-Khan A, Furman RR, O'Brien S, Yenerel MN, Illés A, Kay N, Garcia-Marco JA, Mato A, Pinilla-Ibarz J, Seymour JF, Lepretre S, Stilgenbauer S, Robak T, Rothbaum W, Izumi R, Hamdy A, Patel P, Higgins K, Sohoni S, Jurczak W
J Clin Oncol 2021 Nov 1;39(31):3441-3452. Epub 2021 Jul 26 doi: 10.1200/JCO.21.01210. PMID: 34310172Free PMC Article
Eichhorst B, Robak T, Montserrat E, Ghia P, Niemann CU, Kater AP, Gregor M, Cymbalista F, Buske C, Hillmen P, Hallek M, Mey U; ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2021 Jan;32(1):23-33. Epub 2020 Oct 19 doi: 10.1016/j.annonc.2020.09.019. PMID: 33091559
Baliakas P, Jeromin S, Iskas M, Puiggros A, Plevova K, Nguyen-Khac F, Davis Z, Rigolin GM, Visentin A, Xochelli A, Delgado J, Baran-Marszak F, Stalika E, Abrisqueta P, Durechova K, Papaioannou G, Eclache V, Dimou M, Iliakis T, Collado R, Doubek M, Calasanz MJ, Ruiz-Xiville N, Moreno C, Jarosova M, Leeksma AC, Panayiotidis P, Podgornik H, Cymbalista F, Anagnostopoulos A, Trentin L, Stavroyianni N, Davi F, Ghia P, Kater AP, Cuneo A, Pospisilova S, Espinet B, Athanasiadou A, Oscier D, Haferlach C, Stamatopoulos K; ERIC, the European Research Initiative on CLL
Blood 2019 Mar 14;133(11):1205-1216. Epub 2019 Jan 2 doi: 10.1182/blood-2018-09-873083. PMID: 30602617Free PMC Article
Wiernik PH
Curr Treat Options Oncol 2015 Feb;16(2):8. doi: 10.1007/s11864-014-0323-3. PMID: 25762123
Byrd JC, Brown JR, O'Brien S, Barrientos JC, Kay NE, Reddy NM, Coutre S, Tam CS, Mulligan SP, Jaeger U, Devereux S, Barr PM, Furman RR, Kipps TJ, Cymbalista F, Pocock C, Thornton P, Caligaris-Cappio F, Robak T, Delgado J, Schuster SJ, Montillo M, Schuh A, de Vos S, Gill D, Bloor A, Dearden C, Moreno C, Jones JJ, Chu AD, Fardis M, McGreivy J, Clow F, James DF, Hillmen P; RESONATE Investigators
N Engl J Med 2014 Jul 17;371(3):213-23. Epub 2014 May 31 doi: 10.1056/NEJMoa1400376. PMID: 24881631Free PMC Article

Clinical prediction guides

Hallek M
Am J Hematol 2019 Nov;94(11):1266-1287. Epub 2019 Oct 4 doi: 10.1002/ajh.25595. PMID: 31364186
Fischer K, Al-Sawaf O, Bahlo J, Fink AM, Tandon M, Dixon M, Robrecht S, Warburton S, Humphrey K, Samoylova O, Liberati AM, Pinilla-Ibarz J, Opat S, Sivcheva L, Le Dû K, Fogliatto LM, Niemann CU, Weinkove R, Robinson S, Kipps TJ, Boettcher S, Tausch E, Humerickhouse R, Eichhorst B, Wendtner CM, Langerak AW, Kreuzer KA, Ritgen M, Goede V, Stilgenbauer S, Mobasher M, Hallek M
N Engl J Med 2019 Jun 6;380(23):2225-2236. Epub 2019 Jun 4 doi: 10.1056/NEJMoa1815281. PMID: 31166681
Strati P, Jain N, O'Brien S
Mayo Clin Proc 2018 May;93(5):651-664. doi: 10.1016/j.mayocp.2018.03.002. PMID: 29728204
Scarfò L, Ferreri AJ, Ghia P
Crit Rev Oncol Hematol 2016 Aug;104:169-82. Epub 2016 Jun 16 doi: 10.1016/j.critrevonc.2016.06.003. PMID: 27370174
Wierda WG, Kipps TJ
Curr Opin Hematol 1999 Jul;6(4):253-61. doi: 10.1097/00062752-199907000-00010. PMID: 10400375

Recent systematic reviews

Isaksen IM, Dankel SN
Clin Nutr 2023 Jun;42(6):919-928. Epub 2023 Mar 30 doi: 10.1016/j.clnu.2023.03.018. PMID: 37087831
Lai M, Pampena R, Cornacchia L, Odorici G, Piccerillo A, Pellacani G, Peris K, Longo C
Int J Dermatol 2022 May;61(5):548-557. Epub 2021 Aug 5 doi: 10.1111/ijd.15813. PMID: 34351635Free PMC Article
Delestre F, Blanche P, Bouayed E, Bouscary D, Mouthon L, Brezin A, Le Jeunne C, Chaigne B
Surv Ophthalmol 2021 Jan-Feb;66(1):124-131. Epub 2020 May 11 doi: 10.1016/j.survophthal.2020.05.001. PMID: 32407752
Egle A, Pleyer L, Melchardt T, Hartmann TN, Greil R
Cancer Treat Rev 2018 Nov;70:56-66. Epub 2018 Aug 10 doi: 10.1016/j.ctrv.2018.08.003. PMID: 30121491
Hodgson K, Ferrer G, Montserrat E, Moreno C
Haematologica 2011 May;96(5):752-61. Epub 2011 Jan 17 doi: 10.3324/haematol.2010.036152. PMID: 21242190Free PMC Article

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