Lymphedema classification

 

Depending on etiology, lymphedema is largely divided into primary and secondary lymphedema.

Primary lymphedema

- Congenital lymphedema: Swelling is present from birth.

- Lymphedema praecox: Develops after birth and before the age of 35.

- Lymphedema tarda: Develops from the age of 35.

Primary lymphedema arises from defective lymphatic circulation and impaired absorption of the lymph fluid due to congenital aplasia, hypoplasia and hyperplasia of the lymphatic duct. Lymphedema praecox takes up 75% of all cases of primary lymphedema, among which 75% affect post-pubertal young women.

The cause of primary lymphedema was found to be abnormality in the FOXC2 and VEGF-C genes. A third gene abnormality is also suspected but has not been clarified. Primary lymphedema affects one in every 6,000 people.

 

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Secondary or acquired lymphedema

Secondary or acquired lymphedema most frequently develops due to surgical treatment of cancer or radiotherapy. It most frequently affects patients with breast or uterine cancer.

- Incidence is 15-20% after mastectomy or lumpectomy removing lymph nodes.

- Incidence is 50-70% after surgical removal of lymphatic duct combined with radiotherapy.

- Secondary lymphedema can occur even 30 years after surgery.

It can also develop due to post-surgical strenuous exercise, long-term travel, or excessive stress.

 

Conditions known to be etiologically related to secondary lymphedema

Infection: Culex, brugia timori, aedes aegypti, anopheles, mansonia, and wuchereria bancrofti which are common in the tropics; filiarisis infection (nematoda wuchereria bancrofti, brugia malayi, brugia timori) caused by moisquitoes; tuberculosis, toxoplasmosis infection, nonspecific cellulitis, etc.

Tissue damage: surgical removal of lymph nodes; lymph node removal for biopsy; damaged lymphatic duct or lymph nodes; burn deep enough to damage lymph nodes; radiotherapy in areas of many lymph nodes; patients with breast cancer, malignant melanoma in the limb, prostatic cancer, testicular cancer, uterine cancer, or ovarian cancer receiving lymph node removal or radiotherapy.

Malignant tumor: malignant lymphomatoid, Kaposi sarcoma, metastatic cancer, malignant mass obstructing the veins or lymphatic system in patients with metastasis in the lower abdomen.

Inflammation: lymphadenopathy, bacterial or fungal infection, cellulitis, severe inflammation including erysipelas; immunodeficiency, rheumatism, psoriasis, granuloma.

Artificial cause: excessive tightening of the tourniquet due in kidney diseases, etc.; artificial lymphedema.

Immobility: hyteria-related immobility.

Venous disease: post thrombotic syndrome, chronic venous ulcer.

Morbid obesity: Other risk factors include older age, abnormal nutrition, severe obesity, etc. Presence of diabetes, renal impairment, and heart disease can worsen edema.

 

-To be continued-