Haematology

Subject-specific details about the most common form of leukaemia

Leukaemia is a malignant disease of the blood, characterised by altered haematopoietic progenitor cells and diffuse infiltration of the bone marrow.1

In 2020, leukaemia accounted for around 2.4% of all new cancer cases worldwide and was the cause of over 300,000 deaths.2 In Switzerland, around 1,200 people are diagnosed each year and the 5-year survival rate is 54%.3

Chronic lymphocytic leukaemia (CLL) accounts for 25-30% of all leukaemia cases worldwide4 and mainly affects older people: the average age at diagnosis is 72 years in Switzerland.5 The incidence of CLL is about 2 times higher in men than in women.4 While the mortality rate and survival in Switzerland have improved significantly in recent decades5, the number of people living with lymphocytic leukaemia has risen to 4,370.6

Risk factors for leukaemia include smoking, exposure to certain chemicals, a history of chemotherapy, exposure to radiation, rare congenital diseases, certain blood disorders, family history, age and gender.1 Genetic predisposition is the most important risk factor for CLL. Genetic factors are responsible for around 35% of CLL cases.7

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Survival

The 5-year survival rate for CLL increased from 77.9% (1997-2001) to 83.6% (2012-2016) in Switzerland5

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Risk factors

The most important risk factor for CLL is genetic predisposition7

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Epidemiology

CLL mainly affects older people; men are affected twice as often as women4

Symptoms

CLL patients are usually clinically asymptomatic at the time of diagnosis.8

Overall, 1/3 of CLL patients do not require therapy, 1/3 require therapy during the course of the disease, and 1/3 need therapy at the time of diagnosis.9 The disease is characterized by blood lymphocytosis, which is often detected incidentally.8

As the disease progresses, the following symptoms may occur or worsen:8

  • Anemia/thrombocytopenia
  • Splenomegaly
  • Hepatomegaly
  • Lymphadenopathy
  • Signs of bone marrow insufficiency
  • Refractory autoimmune cytopenia
  • Increased susceptibility to infections
  • Unintentional weight loss
  • Fever
  • Night sweats

Diagnosis

Diagnosis is based on a differential blood count and immunophenotyping of the lymphocytes.8 The following criteria must be met to diagnose CLL:8

  • Detection of at least 5 × 109 clonal B lymphocytes per litre in the peripheral blood over a period of at least three months
  • Predominance of small, morphologically apparently mature lymphocytes in the cytological examination of the blood smear
  • Co-expression of the B-cell antigens CD19, CD20 and CD23 with the T-cell antigen CD5

Treatment

For asymptomatic patients, clinical observation is the standard therapy.8

For patients with symptomatic disease, first-line treatment consists of a therapy that includes either a BTK inhibitor or a BCL2 inhibitor.8 In patients for whom therapy with BTK inhibitors and BCL2 inhibitors is contraindicated, consideration may be given to chemo-immunotherapy with FCR (<65 years) or BR (>65 years), especially in patients with low risk.9

BCL2: B-cell leukaemia/lymphoma 2; BTK: Bruton’s tyrosine kinase; CLL: chronic lymphocytic leukaemia; del(17p): deletions of the short arm of chromosome 17; Hb: haemoglobin; IGVH: immunoglobulin heavy chain variable region.

References:

  1. Huang J, et al. Disease Burden, Risk Factors, and Trends of Leukaemia: A Global Analysis. Front Oncol. 2022 Jul 22;12:904292. 
  2. Bray F, et al. Global cancer statistics 2022: GLOBOCAN estimates of incidence and mortality worldwide for 36 cancers in 185 countries. CA Cancer J Clin. 2024 May-Jun;74(3):229-263. 
  3. Cancer in Switzerland: key figures; Swiss Cancer League, last revised in December 2023. Available online at: https://www.krebsliga.ch/ueber-krebs/zahlen-fakten/-dl-/fileadmin/downloads/sheets/zahlen­krebs-in-der-schweiz.pdf. Last accessed on: 22/04/2024. 
  4. Yao Y, et al. The global burden and attributable risk factors of chronic lymphocytic leukaemia in 204 countries and territories from 1990 to 2019: analysis based on the global burden of disease study 2019. Biomed Eng Online. 2022 Jan 11;21(1):4. 
  5. Andres M, et al. Trends of incidence, mortality and survival for chronic lymphocytic leukaemia / small lymphocytic lymphoma in Switzerland between 1997 and 2016: a population-based study. Swiss Med Wkly. 2021 Mar 15;151:w20463.
  6. Swiss Cancer Report 2021, Federal Office of Public Health, published on 14/10/2021, last updated on: 31/05/2022. Available online at: https://www.bfs.admin.ch/asset/de/19305696.
Last accessed on: 22/04/2024. 
  7. Brown JR. Clinical Risks for Chronic Lymphocytic Leukemia. J Natl Compr Canc Netw. 2024 Apr;22(3):e247020. 
  8. Shadman M. Diagnosis and Treatment of Chronic Lymphocytic Leukemia: A Review. JAMA. 2023 Mar 21;329(11):918-932. 
  9. Hallek M, et al. iwCLL guidelines for diagnosis, indications for treatment, response assessment, and supportive management of CLL. Blood. 2018 Jun 21;131(25):2745-2760. 
  10. Hallek M. Chronic lymphocytic leukemia: 2020 update on diagnosis, risk stratification and treatment. Am J Hematol. 2019 Nov;94(11):1266-1287.

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