Epidemiology in B-Cell Malignancies

¹

Demographics

In the United States, B-cell lymphomas comprise about 85% of all NHL. Regarding these B-cell lymphomas, diffuse large B-cell lymphoma (DLBCL) has the highest incidence of 30%, followed by follicular lymphoma (20%).²More than 70, 000 new cases and almost 19,000 deaths are expected as a result of NHL in 2014.³An estimated 15,720 new cases of CLL and 4600 deaths are expected in 2014.⁴NHL and leukemia are the seventh and eleventh commonest neoplasms, respectively, in the US, with CLL accounting for about one third of leukemia cases.^3-5

• Overall, B-cell NHL and CLL occur in older adults
• Median age at diagnosis is 66 years for NHL and 72 years for CLL, which is rarely diagnosed in individuals under 40 years of age^3,4
• Generally, incidence is higher in men and Caucasians^3,6

Risk Factors^6,7

Common general risk factors for B-cell NHL and CLL are:

• Most patients >60 years of age
• More common in men
• Rare in Asian populations, most found in Caucasians in North America and Europe
• Chemical and radiation exposure may be causes, but more evidence is needed

Differences in risk factors:

• Immunosuppressed transplant patients are at risk for NHL
• Rheumatoid arthritis and systemic lupus erythematosus are associated with higher rates of NHL
• Infections
  • Hepatitis C virus (HCV) is frequently associated with DLBCL and marginal zone lymphoma.⁸Infection with human immunodeficiency virus (HIV) is also associated with the development of NHL, while Epstein-Barr virus (EBV) is associated with DLBCL and other lymphomas⁹
  • Helicobacter pyloriare thought to cause mucosa-associated lymphoid tissue lymphoma (MALT) in the stomach
• Diet may play a role in NHL
• The risk of developing CLL is not thought to be influenced by infections, smoking, or diet
• First-degree relatives of patients with CLL have better than twice the risk of developing CLL

Survival

• Overall 5-year relative survival for patients with NHL is 69.3%, with a median age at death of 76 years³
• 5-year relative survival is influenced by stage at diagnosis³
  • 28% NHL are localized, with a 5-year survival of 81.6%
  • 49% NHL are distant, with a 5-year relative survival of 61.6%
• Specific 5-year survival rates (all races, both sexes, all ages)¹⁰:
  • DLBCL—60.4%
  • DLBCL (not otherwise specified)—60.3%
  • Follicular lymphoma — 86.1%; 1 in 3 cases transitions to a more aggressive B-cell lymphoma²
• 5-year survival for CLL is 79%, and also varies with stage⁴

Characteristics of Some Rarer B-cell Malignancies²

• Primary mediastinal B lymphoma (subtype of DLBCL) occurs more frequently in women 30 to 40 years of age and comprises about 2% of lymphomas
• Burkitt lymphoma, very rare, occurs most commonly in the jawbones of children in Africa but presents as an abdominal mass in US patients
• Small lymphocytic lymphoma accounts for 5% to 10% of all lymphomas and is closely related to CLL, except that the malignant cells are located in the spleen and lymph
• Survival rates for rarer forms¹⁰
  • Small lymphocytic lymphoma—-79.2% (closely related to CLL)
  • Mediastinal B-cell lymphoma—81.8%
  • Mantle-cell lymphoma—55.6%
  • Burkitt lymphoma—58.3%
  • Lymphoplasmacytic lymphoma—-74%

Interesting Facts¹¹

• In the US, a case of blood cancer is diagnosed every 3 minutes
• Six people per hour die from blood cancer in the US
• There are 33% more men than women living with leukemia and more men die of leukemia than women
• More than 500,000 people in the US are either living with active NHL or are in remission
• Age-adjusted incidence of NHL rose by 89.5% from 1975 to 2010

References

1. SEER Survival Monograph; Chapter 28.
2. http://www.cancer.org/cancer/non-hodgkinlymphoma/detailedguide/non-hodgkin-lymphoma-types-of-non-hodgkin-lymphoma. Accessed June 2, 2014.
3. http://seer.cancer.gov/statfacts/html/nhl.html. Accessed June 2, 2014.
4. http://www.cancer.org/cancer/leukemia-chroniclymphocyticcll/detailedguide/leukemia-chronic-lymphocytic-key-statistics. Accessed June 2, 2014.
5. http://seer.cancer.gov/statfacts/html/leuks.html. Accessed June 2, 2014.
6. http://www.cancer.org/cancer/leukemia-chroniclymphocyticcll/detailedguide/leukemia-chronic-lymphocytic-risk-factors. Accessed June 9, 2014.
7. http://www.cancer.org/cancer/non-hodgkinlymphoma/overviewguide/non-hodgkin-lymphoma-overview-what-causes. Accessed June 2, 2014.
8. Peveling-Oberhag J, Arcaini L, Hansmann ML, Zeuzem S. Hepatitis C-associated B-cell non-Hodgkin lymphomas. Epidemiology, molecular signature and clinical management.J Hepatol. 2013;59:169-177.
9. Martelli M, Ferreri AJ, Agostinelli C, Di Rocco A, Pfreundschuh M, Pileri SA. Diffuse large B-cell lymphoma.Crit Rev Oncol Hematol. 2013;87:146-171.
10. http://seer.cancer.gov/csr/1975_2011/results_single/sect_19_table.29_2pgs.pdf. Accessed June 2, 2014.
11. http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/generalcancer/pdf/facts.pdf. Accessed June 5, 2014.