Relate the function of each lymph system component to the formation of lymphedema

P r i m a r y L y m p h e d e m a C a u s e s O n s e t S e c o n d a r y L y m p h e d e m a C a u s e s O n s e t

Inherited genetic mutations resulting in abnormal development of lymph vessels: Hypoplasia Hyperplasia Aplasia Kinmonth syndrome (inguinal lymph node fibrosis)

Genetic mutations with autosomal dominant pattern: Chromosome 5q34q35 FLT4 FOXC2 VEGFR3 SOX18 Variable age onset depending upon the gene involved* Damage to lymph nodes and/or lymph vessels that results in decreased lymphatic reabsorption and transportation (mechanical insufficiency) Trauma Radiation therapy Tumor obstruction Infection CVI Any time after damage to lymph nodes and vessels: Either soon after lymph node dissection or months, years, or decades later Depends on patient medical history, comorbidities (number of lymph nodes removed, radiation therapy, chemotherapy, venous pathologies, and obesity), and general health

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Milroy's disease (congenital lymphedema) FOXC2 VEGEFR3 SOX18 Onset during infancy*

Meige's disease (lymphedema praecox) MIM153200 Onset in childhood or around puberty; may begin in early 20s or 30s

Lymphedema tarda Onset at 35 years or later Surgery Trauma Radiation therapy Tumor obstruction (cancer) Infection CVI Depends on patient medical history, comorbidities, and general health

* Primary and secondary lymphedema differ in causes and onset, which are used to make a differential diagnosis; however, the characteristics are very similar—an edematous limb, skin changes, loss of joint spaces. There are some characteristics of hereditary lymphedema, however, that distinguish it from secondary lymphedema. These include but are not limited to hypoparathyroidism, microcephaly, intestinal lymphangiectasia, ptosis, yellow nails, pleural effusions, cerebral arteriovenous anomalies, distichiasis, congenital heart defects, and webbing of the neck.

Data from Connell F, Brice G, Jeffery S, Keeley V, Mortimer P, Mansour S. A new classification system for primary lymphatic dysplasias based on phenotype.Clin Genet. 2010;77:438–452; Levinson KL, et al. Age of onset in hereditary lymphedema. J Pediatr. 2003;142:704–708; Rizzo C. Lymphedema praecox. Derm Online J. 2009;15(8):7.

The lymphatic system is interrelated with all of the other systems of the body. Its primary roles include conducting immune system surveillance, assisting the cardiovascular system to maintain fluid homeostasis, and aiding the digestive system in the breakdown of longchain fatty acids. The immunological Downloaded 2022323 7:53 A Your IP is 165.84.189.140 functions involve both the immediate response to pathogens and the longterm resistance to repeated exposure to pathogens. Chapter 5: Lymphedema, Marisa Perdomo; Rose L. Hamm ©2022 McGraw Hill. All Rights Reserved. Terms of Use • Privacy Policy • Notice • Accessibility