Disorders of the Lymphatic System

FNK 202

Brief Overview of lymphatic System

The lymphatic system consists of lymph vessels, the lymph nodes and nodules, the spleen and the thymus. Functions of the lymph system are to return tissue fluid to maintain blood volume and to protects the body against pathogens and other foreign material.

Hodgkin's Disease
Hodgkin's disease is a lymphoma, which is cancer of the lymph. Also known as malignant disorder of lymph nodes. Distinguishing feature is Reed-Sternberg cells, which make it different from all the other forms of lymphomas.

Etiology
Unknown Is said to be from a possible virus origin. Most common in people who have mononucleosis. Sometimes occurs in families, suggesting a genetic link. More prevalent in men and occurs most often in young adults ages 15-40 and peaks again in adults older then 55.

Pathophysiology
HD begins as a single changed lymph node, usually in the cervical lymph nodes of the neck. As disease progresses the cancer invades the lymph node chains, node by node. The path of cancer infiltration is usually the same as the path for lymph node flow.

Clinical manifestations
Painless swelling Swelling can range from a size similar to that of a softball, occasionally even larger. Generalized pruritis Low grade fever Night sweats Fatigue weight loss Weakness.

Diagnostic tests
Lymph node biopsy Bone marrow biopsy and aspiration Liver and spleen biopsies Routine chest x-ray Abdominal computed tomography Lymphangiography Hematological tests

Stages of HD testing
Stage 1: Disease is limited to a single lymph node. Stage 2: Two or more nodes are involved on the same side of the diaphragm. Limited organ involvement may ort may not be present.

Stages
Stage 3: is characterized by nodes on both sides of the diaphragm, with or without organ involvement. Stage 4: the most serious form of the disease and the least curable, includes widely disseminated disease in several organs or tissues with or without associated lymph nodes involvement.

Treatment
Radiation Chemotherapy.

Non Hodgkin's Lymphoma

This classification covers all lymphomas other than Hodgkin lymphoma. Predominantly seen in middle life, they are 3 times more common than Hodgkin's lymphoma.

Pathophysiology
Most distinguishing difference is the absence of Reed Sternberg cells in non Hodgkin's lymphoma. Instead many of these lymphomas arise from the B cells and T cells. An abnormality in any of these cells can result in a type of NHL. Most cases of NHL are of B cell origin.

Etiology
The cause of non Hodgkin's lymphomas is unclear, but some viruses, such as Epstein-Barr virus and herpes virus are thought to play a role in their development. Immune problems such as Aids increase risk. Exposure to nuclear waste and some toxic chemicals may also increase risk.

Signs and symptoms

Clinical features of malignant lymphomas include: enlarged rubbery nodes in the cervical and supraclavicular areas, enlarged tonsils and adenoids; and occasional symptoms of dyspnea and cough. As disease progresses the patient may report fatigue, malaise, weight loss and night sweats. NHL usually progresses more rapidly than HD

Diagnostic tests
Disease is confirmed by histological evaluation of biopsied lymph nodes, tonsils, bone marrow, liver, bowel, skin or any other affected tissues. Other relevant tests include: Bone scans, chest x-ray, lymphangiography, liver and spleen scan, CT of abdomen and intravenous pyelogram to determine extent of disease.

Treatment
Multimodal therapy-including the use of chemotherapy and radiation given in combination. Stem cell transplants may be tried in patients with advanced staged disease.

Lympho edema
Abnormal accumulation of fluid within the tissues. Caused by an obstruction in flow. Classified as primary or secondary. More common in women.

Primary Lymph edema

Is quite rare. Results from hypo plastic, a plastic or hyper plastic development of the lymphatic vessels.

Secondary Lymphadema
Acquired Develops from trauma to lymph nodes.

Common cause
Lymphomas Inflammation Parasitic infection Radiation induced fibrosis Removal of lymph nodes.

Signs and symptoms

Heavy aching limbs Weakness Prone to repeated infections Skin ulcers that are difficult to heal.

Pathophysiology
Includes: Roughening of the surface of the lymphatic vessel Dilation of some lymph channels with thickening and edema of the lymphatic tissue. Fibrosis and separation of elastic fibers that maybe present in inflammatory slates.

Recurrent Episodes
May cause fibrosis and hyperplasia of lymph vessels, leading to a severe enlargement of the extremity called elephantiasis.

Diagnostic tests
Lymphangiography CT scan.

Lymphatic Filariasis
Known as Elephantiasis Is a parasitic and infectious tropical disease, that is caused by thread like filarial nematode worms in the super family Filarioidea also known as filariae.

Some facts
Elephantiasis puts at risk more than a billion people in more than 80 countries Over 120 million have already been affected by it, over 40 million of them are seriously incapacitated and disfigured by the disease. 1/3 of the people infected with the disease live in India, 1/3 are in Africa and most of the remainder are in South Asia, the Pacific and South America.(WHO,2000)

Facts
Because lymphatic filarial is a predominantly a disease of poor urban and rural populations, the lymph edema is often untreated, and develops into irreversible elephantiasis. It is believed that 120 million people are infected with lymphatic filarial worms. (WHO,2000)

Facts
In tropical and subtropical areas where lymphatic filariasis is well established, the prevalence of infection is continuing to increase. Primary cause of this is the rapid and unplanned growth of cites, which creates breeding cites for the mosquitoes that transmit the disease.

Pathophysiology
The thread like parasitic filarial worms Wuchereria brancrofti and Brungia malayi that cause fiariasis live almost exclusively in humans. Elephantiasis result when the bite of an infected mosquitoes transmit a microscopic, thread like filarial worm. These parasites enter the lymphatic vessel and are carried to the regional lymph nodes where they cause a significant swelling resulting in lympoedema.

Transmission
The disease is transmitted by mosquitoes that bite infected humans and pick up the microfilariae that develop, inside the mosquito, into the infective stage, in a process that usually takes 7-21 days. The larvae than migrate to the mosquitoes biting mouth parts, ready to enter the punctured skin following the mosquito bite, thus completing the cycle.

Clinical manifestations
Most spectacular symptom of lymphatic filariasis is elephantiasis- edema with thickening of the skin and underlying tissues. Affects mainly the lower extremities. Wuchereria bancrofti can affect the legs, arms, vulva and breasts. Brugia timori rarely affects the genitals.

Diagnosis
Filariasis is usually diagnosed by identifying microfilariae on a Giemsa stained thick blood film.

Treatment
Drugs-The tablet albendazole and DEC have shown to be effective in killing the adult stage filarial parasites. Treatment for chronic lymphoedema include manual lymphatic drainage (MLD) which involves gentle rhythmic massage to encourage the flow of lymph. Complete decongestive therapy (CDT) which incorporates therapeutic exercises

Treatment
Short stretches, compression bandages with skin case and (MLD) and sequential gradient pump therapy, which helps to break up the fibrous tissue, reenabling movement of lymph. Benzopyrones can promote proteolysis (breakdown of protein) permitting fluids to be reabsorbed. (Ganong, 2001).

Own notes on Splenic disorders

I.e. sickle cell anemia and idiopathic thrombocytopenia. Splenomegaly Splenectomy.

References
. Cahill,K.M., Giles, H.M. (2001).Tropical medicine- A clinical text. Shelton CT:Le Jacq Communications. Ganong, W. (2001). Review of medical physiology. Maiden head: Williams and Wilkins.

References
WHO.(2000). Lymphatic Filarisis. (Fact Sheet 102). Geneva: WHO. Williams, L.S., Hopper,P.D. (2003). Understanding medical surgical nursing. (2nd ed.).Philadelphia: F.A. Davies Company.