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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
B-cell chronic lymphocytic leukemia: clinical impact of biological prognostic factors and updated treatment strategies.
Brugiatelli M, Loteta B, Nocilli L, Mannina D
Lijec Vjesn 2007 May;129 Suppl 3:26-8. PMID: 18959056
Monoclonal antibody licensed for third-line treatment of B-cell chronic lymphocytic leukemia.
Thompson CA
Am J Health Syst Pharm 2001 Jul 1;58(13):1174. doi: 10.1093/ajhp/58.13.1174. PMID: 11449871

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 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
Chronic lymphocytic leukemia.
Shanshal M, Haddad RY
Dis Mon 2012 Apr;58(4):153-67. doi: 10.1016/j.disamonth.2012.01.009. PMID: 22449365

Diagnosis

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 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
Chronic lymphocytic leukemia.
Munir T
Hematology 2015 May;20(4):245-6. doi: 10.1179/1024533215Z.000000000356. PMID: 25906256
Chronic lymphocytic leukemia.
Shanshal M, Haddad RY
Dis Mon 2012 Apr;58(4):153-67. doi: 10.1016/j.disamonth.2012.01.009. PMID: 22449365

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
Mechanisms of ibrutinib resistance in chronic lymphocytic leukemia and alternative treatment strategies.
Lama TG, Kyung D, O'Brien S
Expert Rev Hematol 2020 Aug;13(8):871-883. Epub 2020 Aug 12 doi: 10.1080/17474086.2020.1797482. PMID: 32673127
Selecting Frontline Therapy for CLL in 2018.
Jain N
Hematology Am Soc Hematol Educ Program 2018 Nov 30;2018(1):242-247. doi: 10.1182/asheducation-2018.1.242. PMID: 30504317Free PMC Article
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
Ofatumumab.
Keating MJ, Dritselis A, Yasothan U, Kirkpatrick P
Nat Rev Drug Discov 2010 Feb;9(2):101-2. doi: 10.1038/nrd3100. PMID: 20118960

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: 37595283Free PMC Article
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

Prognostic and Predictive Implications of Cytogenetics and Genomics.
Ljungström V, Baliakas P
Hematol Oncol Clin North Am 2021 Aug;35(4):703-713. Epub 2021 May 28 doi: 10.1016/j.hoc.2021.04.002. PMID: 34174981
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
Relevance of Prognostic Factors in the Era of Targeted Therapies in CLL.
Kittai AS, Lunning M, Danilov AV
Curr Hematol Malig Rep 2019 Aug;14(4):302-309. doi: 10.1007/s11899-019-00511-1. PMID: 31049852
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

Recent systematic reviews

Outcomes of COVID-19 infection in patients with chronic lymphocytic leukemia: a systematic review and meta-analysis.
Akbarzadeh MA, Vaez-Gharamaleki Y, Jahanshahlou F, Bavil AG, Hamzehzadeh S, Seifimansour S, Rahimi-Mamaghani A, Hosseini MS
Rev Assoc Med Bras (1992) 2024;70(8):e20240322. Epub 2024 Aug 16 doi: 10.1590/1806-9282.20240322. PMID: 39166680Free PMC Article
Efficacy and safety of new-generation Bruton tyrosine kinase inhibitors in chronic lymphocytic leukemia/small lymphocytic lymphoma: a systematic review and meta-analysis.
Yin S, Zheng X, Zhang W, Zhao H, Zhang R, Li W, Chen F
Ann Hematol 2024 Jul;103(7):2231-2244. Epub 2023 Oct 16 doi: 10.1007/s00277-023-05486-x. PMID: 37843620Free 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
Priapism in Lymphoproliferative Disorders: A Systematic Review.
Ali EA, Sardar S, Yassin MA
Hematol Oncol Stem Cell Ther 2022 Dec 23;15(4):176-182. doi: 10.1016/j.hemonc.2021.05.003. PMID: 34157311
Prognostic models for chronic lymphocytic leukemia (CLL): a systematic review and meta-analysis.
Molica S, Giannarelli D
Leukemia 2021 Feb;35(2):615-618. Epub 2020 Jun 21 doi: 10.1038/s41375-020-0924-8. PMID: 32565543

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