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Lymphatic System Diseases

Lymphatic System Diseases

Our environment is always teeming with millions of diverse pathogenic organisms. Our constant interaction with our surroundings makes it impossible for us to avoid them. The lymphatic system evolved, millions of years ago, to help us defend against them. It is, however, prone to many conditions that render it unable to perform its functions properly.
Kanika Khara
Last Updated: Apr 4, 2018
Adenoid. Normal and Enlarged adenoid
The lymphatic system is a network of organs and tissues whose main function is to help us defend ourselves against infections. It consists of lymph nodes, lymphatic vessels and the lymphoid organs. The lymph nodes are found all over the body. Their function is to filter the lymph. The lymph nodes are connected to the lymphatic vessels, which convey lymph to and away from the lymph node. The lymphoid organs are the thymus and spleen. Thymus is the site where immature T lymphocytes come after leaving the bone marrow, and reach maturity. The spleen is the largest lymphoid organ. It performs many functions, such as filtering blood and removing old red blood cells from the circulation.
Lymphadenopathy
Lymphadenopathy is a disease which is characterized by the inflammation of the lymph nodes. This inflammation can be localized to only some nodes or can be generalized. Usually, a localized inflammation of lymph nodes is due to an infection, foreign particles, or other diseases of organs that are situated close to them. Lymph nodes inflamed due to an infection tend to be painful, but those inflamed from cancers are usually painless.
The Site of Inflammation
Enlarged lymph nodes at a particular site are often associated with certain diseases. Axillary lymph nodes can get affected by infections or cancers of the hand, breast and chest region. Supraclavicular lymph nodes can get affected by diseases of the lungs, esophagus and thorax. Inguinal lymph nodes are connected to the lower abdominal region, external reproductive structures, perineum and lower anal region. Diseases of any of these organs can have an impact on the inguinal lymph nodes. Bilateral hilar lymphadenopathy (BHL) occurs when mediastinal lymph nodes are affected. This is a major feature of the disease, sarcoidosis. Likewise, whichever group of nodes is connected to a particular region, it is affected by diseases of that region. Generalized lymphadenopathy, involving the lymphatic system of the whole body, can occur due to HIV infection, tuberculosis and cancers of the lymphatic system.
Causes
Many diseases have lymphadenopathy as a secondary complication. Some such factors are:
Pathogenic Organisms: Infection by various bacteria, viruses, fungi and other organisms can cause inflammation of the lymph nodes. Bacteria (Staphylococci, Streptococci, Mycobacteria, Brucella, etc.), viruses (HIV, Epstein-Barr virus, etc.), fungi (Histoplasma, Coccidioides, etc.), and other organisms like Toxoplasma gondii and Leishmania, commonly cause infections that affect lymph nodes.
Other Causes: Cancers of the lymphoid system or cancers that have originated in a part of the body and metastasized to other locations may cause lymphadenopathy. Other diseases that have lymphadenopathy as a complication are sarcoidosis, rheumatoid arthritis and amyloidosis. Some drugs such as phenytoin can also cause lymph node inflammation.
Lymphoma
Cancers of the lymphoid tissues are referred to as lymphomas. They are grouped into two broad types, known as Hodgkin's lymphoma and Non-Hodgkin's lymphomas, based on a feature that distinguishes the two from each other. That criterion is the presence of a type of large cell, known as Reed-Sternberg cell, in Hodgkin's lymphoma. This cell is not present in the cancerous tissues of Non-Hodgkin's lymphoma; and therefore, is the basis of their segregation into two classes.
Hodgkin's Lymphoma
It is a type of cancer that occurs primarily in the lymph nodes. Based on the appearance of the cancerous tissues in a biopsy, it is grouped into two types, nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) and Classical Hodgkin's lymphoma. The latter is further subdivided into four types, based on the appearance of cells in laboratory findings.
Symptoms
Like most cancers, a person suffering from Hodgkin's lymphoma may or may not manifest all its symptoms. Some patients remain asymptomatic till the disease has reached an advanced stage. Some of the symptoms are:
  • Painless, rubbery, enlarged lymph nodes
  • Unexplained exhaustion and fever
  • Dry cough and breathing problems
  • Severe itching in the legs or in the entire body
  • Profuse nighttime sweating
  • Weight loss
The symptoms of Hodgkin's lymphoma are divided into different stages based on severity of symptoms. The treatment plan of a person suffering from Hodgkin's lymphoma is guided by the stage to which the cancer has progressed. Stage I Hodgkin's lymphoma is defined as that in which the cancer is localized only in a single group of lymph nodes or if only a single organ is affected. In stage II, two or more groups of lymph nodes are affected or a single organ and one or more group of lymph nodes are affected. Stage III lymphoma is one in which the cancer has spread to both sides, above and below the diaphragm. Finally, stage IV cancer is one that has spread to the entire body, and involves many lymph nodes and organs. Additionally, a cluster of symptoms (fever, weight loss and night sweats), called 'B' symptoms are an important criterion for deciding treatment approach.
Treatment
The treatment depends upon the stage of the disease, and involves chemotherapy and radiation therapy. Although many factors determine which treatment option is to be used, generally stage I and stage II are treated with radiation therapy. Patients with 'B' symptoms, those with advanced stage II, stage III and stage IV, are treated with chemotherapy.
Non-Hodgkin's Lymphoma
Non-Hodgkin's lymphoma is a cancer of the lymphoid system. It is divided into three types: high-grade, intermediate-grade and low-grade. High-grade NHL progresses fast and can be life-threatening if left untreated. However, if detected, it is curable. Low-grade NHL, on the other hand, is slow-growing and does not produce symptoms easily. But it is not curable by the methods used for treating cancers.
Symptoms
Many of the symptoms of NHL are similar to those of Hodgkin's lymphoma, like fatigue, weight loss, fever, itching and night sweats. Swollen and painless lymph nodes develop in the neck region, armpits and inguinal region. Persistent coughing may indicate cancer in the thymus. If the cancer spreads to the brain, symptoms like headache, cognitive problems and seizures may be seen. Many NHL patients have abdominal swelling and pain.
Treatment
It is based on whether the cancer is high-grade, intermediate-grade or low-grade and several other factors. The treatment options include radiation therapy, chemotherapy, monoclonal antibody therapy and autologous stem cell transplantation.
Lymphangitis
Lymphangitis is an inflammation of the lymphatics (lymph channels) due to an infection by a microbe or some chemical irritant. It occurs when an infection or inflammation occurs somewhere else and the microbe or the irritant is transported along with lymph fluid through the lymphatics. This leads to a secondary infection and inflammation, distinct from the infection or inflammation at the primary site. The infection affects the lymph vessel walls, which become inflamed. The consistency of the lymph changes and becomes thick. Microbes that cause lymphangitis mostly enter the lymphatics via a skin infection.
Causes
Bacteria such as Streptococci, Staphylococci and even Neisseria can cause lymphangitis. Nodular lymphangitis, which is another form of this disease in which nodules form below the skin, is caused by microbes like Mycobacteria, Nocardia and Sporothrix, Leishmania, Francisella, etc. Additionally, cancer cells can colonize the lymph vessels and block them.
Symptoms
Red streaks or lines are visible on the skin, charting the course of the inflammation, which runs along the inflamed lymphatic vessels to the closest lymph node. This is accompanied with fever, pain, swelling and tenderness.
Treatment
The primary infection due to which lymphangitis has occurred is treated, usually with antibiotics. If not treated, the infection may spread to the blood stream, causing septicemia, which is a life-threatening condition. If lymphatic fluid is trapped, it may be drained by an incision. A bandage is applied if the lymphangitis has affected an extremity.
Lymphangiectasia
Dilatation of the lymph vessels is known as lymphangiectasia. It can occur as a primary disorder, wherein the cause of the disorder is a congenital defect, i.e. the lymphatic vessels are joined to each other. This disease can also occur as secondarily, due to an obstruction in the lymph vessel. Such obstruction can occur due to lymphomas or filariasis.
Intestinal Lymphangiectasia: It occurs when the intestinal lymphatic vessels are dilated. The disease is characterized by leaking of protein and fat-containing lymph in the small intestine. Other symptoms include swollen peripheral lymphatics and fat-rich stools. This occurs because of an obstructed lymphatic channel, which leads to poor drainage of lymph.
Pulmonary Lymphangiectasia: It is a disorder that can occur due to several factors, but is most often due to a congenital defect in the lymphatic vessels of the lungs. Children born with this disorder usually die within a few hours after their birth. Those who survive have breathing difficulties, cough a lot and suffer from frequent bouts of pneumonia.
Treatment
If lymphangiectasia is caused by secondary factors, treatment is aimed at those underlying causes. Congenital intestinal lymphangiectasia can be managed by modifying the diet. A diet low in fat and having medium-chain fatty acids is recommended for people suffering from this condition. Medicines to control diarrhea can be taken for managing the motility problems, resulting from this disease.
Lymphatic Filariasis
It is perhaps the most well-known disease of the lymphatic system because of the severe disfigurement of affected body parts that it causes. It occurs when mosquitoes that harbor the larvae of certain nematodes bite a person. The larvae enter the lymphatic system, where they mature into adult worms. These adult worms produce many larvae during their lifetime, that mature into adult worms, continuing the cycle. The nematodes obstruct the lymphatic vessels, leading to enlargement of body parts like legs and scrotum.
Causes
It is caused by nematodes like Wuchereria bancrofti, Brugia malayi and Brugia timori. These nematodes are transmitted by mosquitoes of the genera Culex, Anopheles and/or Aedes.
Symptoms
Symptoms include inflammation of the skin and lymph nodes. The damage to the lymphatic system leads to impaired defense against other infections, which is the reason behind the inflammation. Chronic infection causes thickening of tissues and accumulation of lymph. All these cause the massive swelling of organs called elephantiasis.
Treatment
An oral dose of Albendazole along with Ivermectin and Diethylcarbamazine citrate (DEC) is effective in killing mature nematodes as well as the larvae. Eradication of mosquitoes is ideally the best method as it would prevent people from contracting the disease.
Splenomegaly
Splenomegaly is a condition in which the spleen becomes enlarged, tender and painful. It can occur due to a number of reasons, ranging from certain infections to cancers. Many people remain asymptomatic while others have symptoms like tiredness, feeling satiated even after eating a small amount of food, and increased susceptibility to infections.
Causes
Bacterial, viral and parasitic infections, pathological conditions of the liver, hematological diseases, autoimmune disorders and several kinds of cancers can cause splenomegaly.
Treatment
In theory, treatment of the underlying conditions causing the enlarged spleen, will treat the splenomegaly as well. If this is not possible, radiation therapy, or surgical removal of the spleen are the options available to those suffering from the condition.
It is an aphorism in biology that the more complex a system, the greater the price that an organism has to pay for its acquisition. The numerous diseases that the lymphatic system is susceptible to, illustrate it quite clearly.
Disclaimer: This article is for informative purposes only, and should not be used as a replacement for expert medical advice.