Epidemiology

Although probably underestimated, approximately 140 million to 250 million people worldwide have lymphoedema.[6][7]​ In spite of this, adequate healthcare provision for lymphoedema is currently lacking in the majority of countries worldwide.[8]​ Secondary lymphoedema is the most common form of lymphoedema with an estimated prevalence of 1 in 1000 people.[5]

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]

In developed countries, treatment for cancer (e.g., lymphatic resection, irradiation) is the most common cause.[10] 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][12]​​​​ 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] Following breast cancer treatment, the risk of lymphoedema is proportional to the extent of treatment and the cancer stage.[11][12]​ 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] 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]

Primary lymphoedema is rare; the prevalence has been estimated at 1.15 per 100,000 in children.[2][16] Males and females are affected equally; however, males typically present in infancy, whereas females typically present in adolescence.[3] The incidence of primary lymphoedema in adults is difficult to discern because of temporal overlap with secondary lymphoedema.[17]

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