Abstract
Chronic lymphocytic leukemia is a disease of the elderly, and despite major advances in treatment, remains incurable. The Cancer Registry of Norway has registered data on patients with chronic lymphocytic leukemia since 1953. We aimed to analyze trends in incidence and survival of chronic lymphocytic leukemia in Norway. We identified 7664 patients reported with chronic lymphocytic leukemia to the registry between 1953 and 2012. We gathered information on sex, age at diagnosis, date of death and basis for diagnosis. The age‐standardized incidence increased from 0.6/100.000 person‐years in 1953 to 3.1/100,000 person‐years in 2012. We found a significant decrease in median age between 1993–2002 and 2003–2012 (75 vs. 72 years, 95%CI: 2.52–3.98, P < 0.001). Men were diagnosed at a significantly younger age than women. Immunophenotyping has become the most important diagnostic method after 2002. Median observed survival increased from 3 years in 1952–1963 to 8.5 years in 2003–2012. Five‐ and 10‐year age‐standardized net survival increased throughout the whole period across age groups and reached 79% and 57%, respectively. Median observed survival was significantly shorter in men than in women in 1993–2002 (4.9 vs. 6.1 years, P < 0.001). The gap between survival rates for men and women was diminishing in 2003–2012 in patients younger than 60 years while it remained considerable in older patients. Despite an aging Norwegian population, chronic lymphocytic leukemia (CLL) patients become younger at diagnosis. A fourfold increase in incidence, a prolonged survival, and major changes in diagnostic methods in Norway were observed.
Highlights
Chronic lymphocytic leukemia (CLL) is a blood cancer with clonal proliferation and accumulation of mature B-cells characterized by expression of cell surface antigens CD5, CD19, CD 20, CD 23 and CD 79b and either κ or λ light chains.[3, 4] chronic lymphocytic leukemia (CLL) is considered to be the most common leukemia in the western world.[3]CLL in context of all cancers in NorwayIn 2017, totally 33 564 new cases of cancer diagnosis were registered in Norway and 327 (0.97 %) of these were new cases of CLL
Median survival after second hematologic malignancy (SHM) was 0.8 in treated and 6.4 years in treatment-naïve patients ● We found a significantly younger age at CLL diagnosis in pretreated vs. treatment naïve patients acquiring MN, Diffuse large B-cell lymphoma (DLBCL) or HL in 84 (92% of 91) patients with known CLL treatment history. ● The registration of myelodysplastic syndrome prior 2003 was not obligatory in Norway and mostly based on general codes
We found that patients diagnosed with CLL between 2003 and 2012 had more than doubled the risk of Richter syndrome compared to patients diagnosed between 19932002, and a similar trend was seen at MD Anderson Cancer Center. [204]
Summary
Chronic lymphocytic leukemia (CLL) is a blood cancer with clonal proliferation and accumulation of mature B-cells characterized by expression of cell surface antigens CD5, CD19 , CD 20, CD 23 and CD 79b and either κ or λ light chains.[3, 4] CLL is considered to be the most common leukemia in the western world.[3]. In 2017, totally 33 564 new cases of cancer diagnosis were registered in Norway and 327 (0.97 %) of these were new cases of CLL. In CLL, clonal B-lymphocytes truly proliferate without a specific invader being present and CLL cells are resistant to apoptosis.[8] In addition, clonal B-cells lack the proper ability of intercellular communication, a crucial process in complex functions of the immune system. Chronic lymphocytic leukemia (CLL) is the most common leukemia in Caucasian adults, and some of the highest incidence rates are reported from Scandinavia. The Norwegian population is aging and because the median age at CLL diagnosis has been reported to be about 70 years, the incidence of CLL has been expected to increase
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