Although probably underestimated, approximately 140 million to 250 million people worldwide have lymphoedema.[6]Lopez M, Roberson ML, Strassle PD, et al. Epidemiology of lymphedema-related admissions in the United States: 2012-2017. Surg Oncol. 2020 Dec;35:249-53.
http://www.ncbi.nlm.nih.gov/pubmed/32932222?tool=bestpractice.com
[7]Douglass J, Kelly-Hope L. Comparison of staging systems to assess lymphedema caused by cancer therapies, lymphatic filariasis, and podoconiosis. Lymphat Res Biol. 2019 Oct;17(5):550-6.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6797069
http://www.ncbi.nlm.nih.gov/pubmed/30789319?tool=bestpractice.com
In spite of this, adequate healthcare provision for lymphoedema is currently lacking in the majority of countries worldwide.[8]Schulze H, Nacke M, Gutenbrunner C, et al. Worldwide assessment of healthcare personnel dealing with lymphoedema. Health Econ Rev. 2018 Apr 16;8(1):10.
https://healtheconomicsreview.biomedcentral.com/articles/10.1186/s13561-018-0194-6
http://www.ncbi.nlm.nih.gov/pubmed/29663122?tool=bestpractice.com
Secondary lymphoedema is the most common form of lymphoedema with an estimated prevalence of 1 in 1000 people.[5]Brix B, Sery O, Onorato A, et al. Biology of lymphedema. Biology (Basel). 2021 Mar 25;10(4):261.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8065876
http://www.ncbi.nlm.nih.gov/pubmed/33806183?tool=bestpractice.com
The major single cause is nematode infection (filariasis), which, despite recent improvements, is estimated to cause more than 16 million cases of lymphoedema in developing countries. The aim of the World Health Organization (WHO) is to eradicate filariasis via the coordination of a mass drug delivery programme; as of 2018, there were approximately 51 million people in mosquito-endemic regions with lymphatic filariasis, representing a 74% decline since the start of the WHOs mass drug delivery programme in 2000.[9]Local Burden of Disease 2019 Neglected Tropical Diseases Collaborators. The global distribution of lymphatic filariasis, 2000-18: a geospatial analysis. Lancet Glob Health. 2020 Sep;8(9):e1186-94.
https://www.thelancet.com/journals/langlo/article/PIIS2214-109X(20)30286-2/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/32827480?tool=bestpractice.com
In developed countries, treatment for cancer (e.g., lymphatic resection, irradiation) is the most common cause.[10]Manrique OJ, Bustos SS, Ciudad P, et al. Overview of lymphedema for physicians and other clinicians: a review of fundamental concepts. Mayo Clin Proc. 2020 Aug 20:S0025-6196(20)30033-1.
https://www.mayoclinicproceedings.org/article/S0025-6196(20)30033-1/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/32829905?tool=bestpractice.com
The incidence of cancer-related lymphoedema varies depending on cancer type and treatment and accurate estimates are limited due to differences in lymphoedema definitions and diagnostic criteria.[11]Bernas M, Thiadens SRJ, Stewart P, et al. Secondary lymphedema from cancer therapy. Clin Exp Metastasis. 2021 May 5.
http://www.ncbi.nlm.nih.gov/pubmed/33950413?tool=bestpractice.com
[12]Rockson SG, Keeley V, Kilbreath S, et al. Cancer-associated secondary lymphoedema. Nat Rev Dis Primers. 2019 Mar 28;5(1):22.
http://www.ncbi.nlm.nih.gov/pubmed/30923312?tool=bestpractice.com
Estimation of lymphoedema in breast cancer survivors is approximately 20% and for survivors of gynaecological, melanoma, and head and neck cancer is between 10% and 40%.[13]Singh B, Disipio T, Peake J, et al. Systematic review and meta-analysis of the effects of exercise for those with cancer-related lymphedema. Arch Phys Med Rehabil. 2016 Feb;97(2):302-15.e13.
http://www.ncbi.nlm.nih.gov/pubmed/26440777?tool=bestpractice.com
Following breast cancer treatment, the risk of lymphoedema is proportional to the extent of treatment and the cancer stage.[11]Bernas M, Thiadens SRJ, Stewart P, et al. Secondary lymphedema from cancer therapy. Clin Exp Metastasis. 2021 May 5.
http://www.ncbi.nlm.nih.gov/pubmed/33950413?tool=bestpractice.com
[12]Rockson SG, Keeley V, Kilbreath S, et al. Cancer-associated secondary lymphoedema. Nat Rev Dis Primers. 2019 Mar 28;5(1):22.
http://www.ncbi.nlm.nih.gov/pubmed/30923312?tool=bestpractice.com
Of 936 women with breast cancer, the prevalence of lymphoedema 5 years after sentinel lymph node biopsy was 5%, compared with 16% in women who underwent sentinel lymph node biopsy followed by axillary lymph node dissection.[14]McLaughlin SA, Wright MJ, Morris KT, et al. Prevalence of lymphedema in women with breast cancer 5 years after sentinel lymph node biopsy or axillary dissection: objective measurements. J Clin Oncol. 2008 Nov 10;26(32):5213-9.
http://www.ncbi.nlm.nih.gov/pubmed/18838709?tool=bestpractice.com
Increasing rates of obesity have lead to greater levels of diagnosis of lymphoedema in a sub-group of people who lack other sources of lymphatic compromise.[15]Mehrara BJ, Greene AK. Lymphedema and obesity: is there a link? Plast Reconstr Surg. 2014 Jul;134(1):154e-60e.
http://www.ncbi.nlm.nih.gov/pubmed/25028830?tool=bestpractice.com
Primary lymphoedema is rare; the prevalence has been estimated at 1.15 per 100,000 in children.[2]Brouillard P, Witte MH, Erickson RP, et al. Primary lymphoedema. Nat Rev Dis Primers. 2021 Oct 21;7(1):77.
http://www.ncbi.nlm.nih.gov/pubmed/34675250?tool=bestpractice.com
[16]Smeltzer DM, Stickler GB, Schirger A. Primary lymphedema in children and adolescents: a follow-up study and review. Pediatrics. 1985 Aug;76(2):206-18.
http://www.ncbi.nlm.nih.gov/pubmed/4022694?tool=bestpractice.com
Males and females are affected equally; however, males typically present in infancy, whereas females typically present in adolescence.[3]Schook CC, Mulliken JB, Fishman SJ, et al. Primary lymphedema: clinical features and management in 138 pediatric patients. Plast Reconstr Surg. 2011 Jun;127(6):2419-31.
http://www.ncbi.nlm.nih.gov/pubmed/21617474?tool=bestpractice.com
The incidence of primary lymphoedema in adults is difficult to discern because of temporal overlap with secondary lymphoedema.[17]Goss JA, Maclellan RA, Greene AK. Adult-onset primary lymphedema: a clinical-lymphoscintigraphic study of 26 patients. Lymphat Res Biol. 2019 Dec;17(6):620-3.
http://www.ncbi.nlm.nih.gov/pubmed/30916606?tool=bestpractice.com