U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

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
Bhowmick M, Auckbarallee F, Edgar P, Ray A, Dasgupta S
Exp Clin Transplant 2016 Feb;14(1):17-21. PMID: 26862819
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: [email protected]
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
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
Shanshal M, Haddad RY
Dis Mon 2012 Apr;58(4):153-67. doi: 10.1016/j.disamonth.2012.01.009. PMID: 22449365

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
Sanford M, McCormack PL
Drugs 2010 May 28;70(8):1013-9. doi: 10.2165/11203850-000000000-00000. PMID: 20481657

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: [email protected]
Ann Oncol 2021 Jan;32(1):23-33. Epub 2020 Oct 19 doi: 10.1016/j.annonc.2020.09.019. PMID: 33091559
Moia R, Boggione P, Mahmoud AM, Kodipad AA, Adhinaveni R, Sagiraju S, Patriarca A, Gaidano G
Expert Opin Ther Targets 2020 Dec;24(12):1239-1250. Epub 2020 Oct 19 doi: 10.1080/14728222.2020.1832465. PMID: 33016796
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

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
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
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
Wierda WG, Kipps TJ
Curr Opin Hematol 1999 Jul;6(4):253-61. doi: 10.1097/00062752-199907000-00010. PMID: 10400375

Recent systematic reviews

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
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
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
Grywalska E, Zaborek M, Łyczba J, Hrynkiewicz R, Bębnowska D, Becht R, Sosnowska-Pasiarska B, Smok-Kalwat J, Pasiarski M, Góźdź S, Roliński J, Niedźwiedzka-Rystwej P
Cells 2020 Nov 2;9(11) doi: 10.3390/cells9112398. PMID: 33147729Free PMC Article
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...