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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.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
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).
See: Feature record | Search on this feature
Cellular immunodeficiency
MedGen UID:
344444
•Concept ID:
C1855204
•
Finding
An immunodeficiency characterized by defective cell-mediated immunity or humoral immunity.
See: Feature record | Search on this feature
Show allHide all

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).
See: Condition Record
Leukemia, chronic lymphocytic, susceptibility to, 2
MedGen UID:
358385
•Concept ID:
C1868683
•
Finding
See: Condition Record
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).
See: Condition Record
B-cell chronic lymphocytic leukemia
Immunodeficiency 36
Leukemia, chronic lymphocytic, susceptibility to, 2

Professional guidelines

PubMed

Effect of CD38 on B-cell function and its role in the diagnosis and treatment of B-cell-related diseases.
Zeng F, Zhang J, Jin X, Liao Q, Chen Z, Luo G, Zhou Y
J Cell Physiol 2022 Jul;237(7):2796-2807. Epub 2022 Apr 29 doi: 10.1002/jcp.30760. PMID: 35486480
Alemtuzumab treatment of multiple sclerosis.
Coles AJ
Semin Neurol 2013 Feb;33(1):66-73. Epub 2013 May 25 doi: 10.1055/s-0033-1343797. PMID: 23709214
The changing paradigm of chronic lymphocytic leukemia management.
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

Chronic lymphocytic leukemia treatment algorithm 2022.
Hampel PJ, Parikh SA
Blood Cancer J 2022 Nov 29;12(11):161. doi: 10.1038/s41408-022-00756-9. PMID: 36446777Free PMC Article
Chronic lymphocytic leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.
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
Chronic lymphocytic leukemia (CLL) treatment: So many choices, such great options.
Sharma S, Rai KR
Cancer 2019 May 1;125(9):1432-1440. Epub 2019 Feb 26 doi: 10.1002/cncr.31931. PMID: 30807655
Chronic lymphocytic leukaemia.
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
Chronic lymphocytic leukaemia.
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

A CAPTIVATEing Analysis for Higher-Risk CLL.
Rogers KA
Clin Cancer Res 2023 Jul 14;29(14):2561-2562. doi: 10.1158/1078-0432.CCR-23-0807. PMID: 37289004Free PMC Article
Chronic Lymphocytic Leukemia.
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
Chronic Lymphocytic Leukemia: Diagnosis and Treatment.
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
Chronic lymphocytic leukaemia.
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
Chronic lymphocytic leukaemia.
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

Therapy

Chronic lymphocytic leukemia: 2022 update on diagnostic and therapeutic procedures.
Hallek M, Al-Sawaf O
Am J Hematol 2021 Dec 1;96(12):1679-1705. doi: 10.1002/ajh.26367. PMID: 34625994
ALPINE: zanubrutinib versus ibrutinib in relapsed/refractory chronic lymphocytic leukemia/small lymphocytic lymphoma.
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
iwCLL guidelines for diagnosis, indications for treatment, response assessment, and supportive management of CLL.
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
CD19-CAR trials.
Ramos CA, Savoldo B, Dotti G
Cancer J 2014 Mar-Apr;20(2):112-8. doi: 10.1097/PPO.0000000000000031. PMID: 24667955Free PMC Article
Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines.
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

Sustained remissions in CLL after frontline FCR treatment with very-long-term follow-up.
Thompson PA, Bazinet A, Wierda WG, Tam CS, O'Brien SM, Saha S, Peterson CB, Plunkett W, Keating MJ
Blood 2023 Nov 23;142(21):1784-1788. doi: 10.1182/blood.2023020158. PMID: 37595283
Acalabrutinib Versus Ibrutinib in Previously Treated Chronic Lymphocytic Leukemia: Results of the First Randomized Phase III Trial.
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
Chronic lymphocytic leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.
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
Cytogenetic complexity in chronic lymphocytic leukemia: definitions, associations, and clinical impact.
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
Familial leukemias.
Wiernik PH
Curr Treat Options Oncol 2015 Feb;16(2):8. doi: 10.1007/s11864-014-0323-3. PMID: 25762123

Clinical prediction guides

Chronic lymphocytic leukemia: 2020 update on diagnosis, risk stratification and treatment.
Hallek M
Am J Hematol 2019 Nov;94(11):1266-1287. Epub 2019 Oct 4 doi: 10.1002/ajh.25595. PMID: 31364186
Venetoclax and Obinutuzumab in Patients with CLL and Coexisting Conditions.
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
Chronic Lymphocytic Leukemia: Diagnosis and Treatment.
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
Chronic lymphocytic leukaemia.
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
Chronic lymphocytic leukemia.
Kay NE, Hamblin TJ, Jelinek DF, Dewald GW, Byrd JC, Farag S, Lucas M, Lin T
Hematology Am Soc Hematol Educ Program 2002:193-213. doi: 10.1182/asheducation-2002.1.193. PMID: 12446424

Recent systematic reviews

Clinical Research in Chronic Lymphocytic Leukemia in Pakistan; A Systematic Review.
Ammad Ud Din M, Shahzad M, Ashraf A, Liaqat H, Jaan A, Anwer F
Medicina (Kaunas) 2023 Aug 17;59(8) doi: 10.3390/medicina59081483. PMID: 37629773Free PMC Article
Molecular-Biology-Driven Frontline Treatment for Chronic Lymphocytic Leukemia: A Network Meta-Analysis of Randomized Clinical Trials.
Rizzuto A, Pirrera A, Gigliotta E, Mancuso S, Vullo C, Camarda GM, Rotolo C, Roppolo A, Spoto C, Gentile M, Botta C, Siragusa S
Int J Mol Sci 2023 Jun 9;24(12) doi: 10.3390/ijms24129930. PMID: 37373078Free PMC Article
Ultra-processed food consumption and cancer risk: A systematic review and meta-analysis.
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
Ophthalmic involvement of chronic lymphocytic leukemia: A systematic review of 123 cases.
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
Remission maintenance treatment options in chronic lymphocytic leukemia.
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

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