LYMPHEDEMA : NOT ONE DISEASE Chronic lymphatic-system failure (lymphedema) ​occurs in 3-5 million Americans across a​ ​wide spectrum of diseases.

of all cases are of all cases are 1-3,6-7 1,2,4,5 10 % Primary (congenital) 90% Secondary (acquired)

Over 40 rare diseases are associated with primary lymphedema including: Secondary cases can be broken into two categories: • Aagenaes Syndrome • Meige Syndrome % • Adams-Oliver Syndrome • Microcephaly-Chorioretinopathy- 22 of all cases are non-cancer related. • C.H.A.R.G.E. Syndrome Lymphedema-Mental Retardation Any significant damage to the lymphatic organ system Syndrome • C.L.O.V.E.S. Syndrome can result in lymphedema. Causes include: • Milroy’s Disease • Carbohydrate Deficient Glycoprotein • Burns • Radiation • (types 1a, 1b, 1h) Mucke Syndrome • Ilio-femoral bypass • Rheumatoid arthritis • • ardio-facial-cutaneous Syndrome  • Infection • Surgery • • Paralysis • Trauma • Choanalatresia-lymphedema Syndrome Nonne-Milroy Disease • • Congenital Lymphedema (non-Milroy’s) Noonan’s Syndrome • Oculo-Dento-Digital Syndrome % • Ectodermal Dysplasia Anhidrotic 68 of all cases are cancer related. • • Immunodeficiency Osteopetrosis Parkes-Weber Syndrome The overall cancer-related incidence rate is 15.5%. • • Lymphedema Syndrome Phelan McDermid Syndrome Specific rates include: • • Fabray’s Disease Prader Willi Syndrome • Breast 40% • Melanoma 16% • • Genital-urinary 10% • Gorham’s Disease Progressive Encephalopathy-Hypsar • Sarcoma 30% -rhythmia-Optic Atrophy Syndrome • Gynecological 20% • Head and neck 4% • Hennekam Syndrome • Protein Losing Enteropathy • Hypotrichosis Lymphedema (associated with numerous forms 1. Journal of the American College of . 2008: 52 (10): 799-806 • Klippel Trenaunay Syndrome of congenital heart disease) 2. Vascular Medicine. 1998: (3): 145-156 • Klippel-Trenaunay-Weber Syndrome •  3. Clinical Genetics. 2010: 77: 438-452 • Lipedema • Spina bifida 4. Cancer. 2010: 116: 5138-49 5. Journal of Clinical Oncology. 2009: 27:(3): 390-7 • Lymphedema Distichiasis Syndrome • Thrombocytopenia with Absent 6. Annals of the New York Academy of Sciences. 2008: 1131: 147-154 • Lymphedema Myelodysplasia Radius Syndrome 7. European Journal of Cancer Care. (Engl.) 1996: 5: 56-59 (Emberger Syndrome) • Trisomy 13,18,21 • Lymphedema Praecox • Turner’s Syndrome • Lymphedema Tarda • Velocardiofacial Syndrome • Lymphedema-Distichiasis • W.I.L.D. Syndrome • Macrocephaly- Malformation • Maffucci Syndrome For more information visit our website LymphedemaTreatmentAct.org