Chronic lymphocytic leukemia

"B-cell CLL" redirects here. For the gene family, see B-cell CLL/lymphoma.
Chronic lymphocytic leukemia
Other namesB-cell chronic lymphocytic leukemia (B-CLL)[1]
Peripheral blood smear showing CLL cells
SpecialtyHematology and oncology
SymptomsEarly: None[2]
Later: Non-painful lymph nodes swelling, feeling tired, fever, weight loss[2]
Usual onsetOlder than 50[3]
Risk factorsFamily history, Agent Orange, certain insecticides[2][4]
Diagnostic methodBlood tests[5]
Differential diagnosisMononucleosis, hairy cell leukemia, acute lymphocytic leukemia, persistent polyclonal B-cell lymphocytosis[5]
TreatmentWatchful waiting, chemotherapy, immunotherapy[4][5]
PrognosisFive-year survival ~88% (US)[3]
Frequency904,000 (2015)[6]
Deaths60,700 (2015)[7]

Chronic lymphocytic leukemia (CLL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).[2][8] Early on, there are typically no symptoms.[2] Later, non-painful lymph node swelling, feeling tired, fever, night sweats, or weight loss for no clear reason may occur.[2][9] Enlargement of the spleen and low red blood cells (anemia) may also occur.[2][4] It typically worsens gradually over years.[2]

Risk factors include having a family history of the disease, with 10% of those who develop CLL having such ancestry.[2][9] Exposure to Agent Orange, certain insecticides, sun exposure, exposure to hepatitis C virus, and common infections are also considered risk factors.[4][9] CLL results in the buildup of B cell lymphocytes in the bone marrow, lymph nodes, and blood.[4] These cells do not function well and crowd out healthy blood cells.[2] CLL is divided into two main types:

  • Those with a mutated IGHV gene
  • Those without.[4]

Diagnosis is typically based on blood tests finding high numbers of mature lymphocytes and smudge cells.[5]

Early-stage CLL in asymptomatic cases responds better to careful observation, as there is no evidence that early intervention treatment can alter the course of the disease.[10] Immune defects occur early in the course of CLL and these increase the risk of developing serious infection, which should be treated appropriately with antibiotics.[10] In those with significant symptoms, chemotherapy, immunotherapy, or chemoimmunotherapy may be used.[4] Depending on the individual's age, physical condition, and whether they have the del(17p) or TP53 mutation, different first line treatments may be offered.[11] As of 2021, BTK inhibitors such as ibrutinib and acalabrutinib are often recommended for first line treatment of CLL.[12] The medications fludarabine, cyclophosphamide, and rituximab were previously the initial treatment in those who are otherwise healthy.[13]

CLL affected about 904,000 people globally in 2015 and resulted in 60,700 deaths.[6][7] In 2021, the estimated incidence of CLL in the United States is 21,250 new cases and 4,320 deaths.[14] The disease most commonly occurs in people over the age of 65, due to the accumulation of genetic mutations that occur over time.[3][15] Men are diagnosed around twice as often as women (6.8 to 3.5 ratio).[16] It is much less common in people from Asia.[4] Five-year survival following diagnosis is approximately 83% in the United States.[3] It represents less than 1% of deaths from cancer.[7]

  1. ^ O'Brien S, Gribben JG (2008). Chronic Lymphocytic Leukemia. CRC Press. p. 19. ISBN 9781420068962.
  2. ^ a b c d e f g h i j "Chronic Lymphocytic Leukemia Treatment". National Cancer Institute. 26 October 2017. Retrieved 19 December 2017.
  3. ^ a b c d "Chronic Lymphocytic Leukemia – Cancer Stat Facts". seer.cancer.gov. Retrieved 9 September 2022.
  4. ^ a b c d e f g h Kipps TJ, Stevenson FK, Wu CJ, Croce CM, Packham G, Wierda WG, et al. (January 2017). "Chronic lymphocytic leukaemia". Nature Reviews. Disease Primers. 3: 16096. doi:10.1038/nrdp.2016.96. PMC 5336551. PMID 28102226.
  5. ^ a b c d Ferri FF (2017). Ferri's Clinical Advisor 2018 E-Book: 5 Books in 1. Elsevier Health Sciences. p. 750. ISBN 9780323529570.
  6. ^ a b Vos, Theo; et al. (October 2016). "Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015". Lancet. 388 (10053): 1545–1602. doi:10.1016/S0140-6736(16)31678-6. PMC 5055577. PMID 27733282.
  7. ^ a b c Wang, Haidong; et al. (October 2016). "Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980–2015: a systematic analysis for the Global Burden of Disease Study 2015". Lancet. 388 (10053): 1459–1544. doi:10.1016/s0140-6736(16)31012-1. PMC 5388903. PMID 27733281.
  8. ^ Boelens J, Lust S, Vanhoecke B, Offner F (February 2009). "Chronic lymphocytic leukaemia". Anticancer Research. 29 (2): 605–615. PMID 19331210.
  9. ^ a b c Cite error: The named reference Hall2018 was invoked but never defined (see the help page).
  10. ^ a b Stilgenbauer S, Furman RR, Zent CS (2015-05-01). "Management of chronic lymphocytic leukemia". American Society of Clinical Oncology Educational Book. American Society of Clinical Oncology. Annual Meeting (35): 164–175. doi:10.14694/EdBook_AM.2015.35.164. PMID 25993154.
  11. ^ Hallek M (November 2019). "Chronic lymphocytic leukemia: 2020 update on diagnosis, risk stratification and treatment". American Journal of Hematology. 94 (11): 1266–1287. doi:10.1002/ajh.25595. PMID 31364186. S2CID 199000131.
  12. ^ Patel K, Pagel JM (April 2021). "Current and future treatment strategies in chronic lymphocytic leukemia". Journal of Hematology & Oncology. 14 (1): 69. doi:10.1186/s13045-021-01054-w. PMC 8074228. PMID 33902665.
  13. ^ Hallek M (September 2017). "Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment". American Journal of Hematology. 92 (9): 946–965. doi:10.1002/ajh.24826. PMID 28782884.
  14. ^ "Key Statistics for Chronic Lymphocytic Leukemia". www.cancer.org. Retrieved 2021-11-29.
  15. ^ "Genes and Cancer". Archived from the original on 2021-11-16.
  16. ^ Grywalska E, Zaborek M, Łyczba J, Hrynkiewicz R, Bębnowska D, Becht R, et al. (November 2020). "Chronic Lymphocytic Leukemia-Induced Humoral Immunosuppression: A Systematic Review". Cells. 9 (11): 2398. doi:10.3390/cells9112398. PMC 7693361. PMID 33147729.