Diseases & disorders of the lymphatic system

Diseases of the lymphatic system can appear to be similar to those of the immune system, as these two systems are closely related. The following sections examine the common diseases and disorders of the lymphatic system.

Lymphoma: hodgkin’s and non-hodgkin’s

Lymphoma is a cancer of the lymphatic and immune systems. Lymphocytes, a type of white blood cell, mutate and reproduce rapidly in lymphoma. The overproduced, diseased lymphocytes crowd out healthy WBCs. This causes the patient to be more susceptible to infections.

There are two main types of lymphoma:

• Hodgkin’s lymphoma: Also called Hodgkin disease. It is characterized by the presence of Reed-Sternberg cells in the blood. This type of cell is specific to Hodgkin’s lymphoma.
• Non-hodgkin’s lymphoma: A collection of all other lymphatic cancers that are not Hodgkin’s lymphoma; this is the more common of the two types.

The cause of lymphoma is not well understood or obvious in most cases. There isn’t a direct link to any one event, chemical exposure, or genetic mutation that can predict the development of lymphoma. However, some factors may increase a person’s risk of developing this type of cancer.

Risk factors for developing Hodgkin’s lymphoma include a family history of lymphoma, being 15 to 30 years old or over age 55, being male, and having had an Epstein-Barr (EBV) infection (infectious mononucleosis [mono]).

Risk factors for developing non-Hodgkin’s lymphoma include being over age 60; being on immunosuppressive therapy or medications; and having had certain infections, such as EBV, HIV, or Helicobacter pylori, which causes stomach and intestinal ulcers.

Signs and symptoms of both Hodgkin’s lymphoma and non-Hodgkin’s lymphoma include painless swelling of lymph nodes (especially in the neck, armpits, or groin), fatigue, fever, chills, night sweats, and unexplained weight loss. Additional symptoms more common with non-Hodgkin’s lymphoma are abdominal swelling or pain, trouble breathing, coughing, or chest pain.

For both types of lymphoma, the diagnostic process is the same. The provider will perform a physical exam, specifically looking for enlarged and nontender lymph nodes, liver, and spleen. Blood and urine tests will be done to rule out other possible disease conditions and infections. Blood tests would likely include a CBC and differential and a CMP. Urinalysis also is likely. Imaging tests may include x-rays, computed tomography (CT), MRI, or positron emission tomography (PET) scans. A lymph node, tissue, or bone marrow biopsy may also be done. The provider can make an accurate diagnosis about the type of lymphoma present and the stage of the disease by using information obtained in a biopsy. Staging cancer is important because it affects the treatment plan the provider will recommend.

Treatment depends on the type and stage of the lymphoma. Not all lymphomas are treated. For instance, some slowly progressing types of non-Hodgkin’s lymphoma are monitored and may not be treated. Depending on the type and stage of the lymphoma, treatment may include chemotherapy, radiation therapy, and stem cell transplantation (bone marrow transplantation). A stem cell transplant is used to replace diseased bone marrow with healthy stem cells. Stem cells can then repopulate the bone marrow with healthy cells. Also, biologic drugs may be used to enhance the patient’s immune system, helping it to destroy lymphoma cells.

Multiple myeloma

Multiple myeloma is a cancer of the white blood cells called plasma cells. Plasma cells are a type of B lymphocyte that produce antibodies to help fight infection. In multiple myeloma, plasma cells reproduce so much that they crowd out healthy blood cells. The malignant plasma cells don’t produce antibodies. Instead, they produce an abnormal protein (M protein) that can cause kidney damage.

This condition is called multiple myeloma because the tumors are found in many or multiple bones. If it occurs in only one bone or area, the tumor is referred to as a plasmacytoma.

The cause of multiple myeloma is unknown, or idiopathic. But for most people, multiple myeloma starts as monoclonal gammopathy of undetermined significance (MGUS). People with MGUS also produce M protein, but at such a low level, it does not harm the body. Risk factors for developing multiple myeloma include being over the age of 60, male gender (blacks are more likely to develop multiple myeloma than whites), and a history of MGUS. This makes developing multiple myeloma more likely.

Signs and symptoms of multiple myeloma may be subtle at first. There may be no symptoms at all in the beginning. Noticeable signs and symptoms include bone pain, especially in the spine, chest, or hips; anemia; loss of kidney function; excessive thirst; nausea; loss of appetite; weight loss; constipation; fatigue; frequent infections; weakness or numbness in the legs; and mental confusion.

Tests that may be used to help diagnose multiple myeloma include blood tests, which may include a CBC and differential, tests for the presence of M protein, and a CMP (includes kidney and liver function tests); urinalysis (M proteins in the urine are called Bence Jones proteins); a bone marrow biopsy; and examination of cells. Imaging tests may include x-ray, MRI, CT, and PET scans. At diagnosis, multiple myeloma will be staged, and a treatment plan will be based on the staged diagnosis.

Treatment for asymptomatic multiple myeloma, MGUS, may not be needed. The provider will want to watch the condition and run periodic blood and urine tests to monitor signs of activity. Treatment for multiple myeloma includes targeted cancer therapy (designed to target specific weaknesses in myeloma cancer cells to kill the cells), biologic therapy, chemotherapy, radiation therapy, and possibly stem cell transplantation.

Lymphedema

Lymphedema is when there is an accumulation of protein-rich fluid that accumulates in the tissue, causing swelling, usually in the arms or legs. This fluid is usually drained by the lymphatic system. The lymph vessels could be compromised by infection, cancer, scar tissue from radiation therapy, or surgical removal of the lymph nodes.

Signs and symptoms of lymphedema include the following:

• Swelling of all or part of the arm or leg
• A feeling of heaviness or tightness
• Restricted range of motion
• Recurring infections
• Hardening and thickening of the skin (fibrosis)

Diagnosis of lymphedema may be made based on the presenting signs and symptoms if the patient is at risk for developing lymphedema. If that is not the case, an MRI, CT, or ultrasound may be done to see if there are obstructions in the lymphatic system.

There is no cure for lymphedema, and treatment focuses on reducing the swelling. Compression bandages or compression garments may be used to encourage lymph fluid drainage. A sequential pneumatic compression sleeve is connected to a pump that intermittently inflates the sleeve using pressure to help move the lymph fluid away from the fingers and toes. There are several surgical procedures that can help, including lymph node transplants, development of new drainage paths, and removal of fibrous tissue that has become hardened.

Tonsillitis

Tonsillitis is the inflammation of the tonsils found at the back of the throat. Most cases of tonsillitis are caused by common viruses. Bacteria can also cause tonsillitis, with Streptococcus pyogenes (group A Streptococcus) being the most common. These bacteria also cause strep throat.

Signs and symptoms of tonsillitis include these:

• Red, swollen tonsils
• Scratchy, muffled or throaty voice
• Sore throat
• Bad breath
• Difficult or painful swallowing
• Stomachache
• Fever
• Neck pain or stiff neck
• Enlarged, tender lymph glands in the neck
• Headache
• White or yellow coating or patches on the tonsils

The throat, nose, and ears will be examined to look for the infection. Palpating the neck for swollen lymph glands, listening to the patient’s breathing with a stethoscope, and checking for enlargement of the spleen will also be done. Lab work could include a throat swab to check for streptococcal bacteria and a CBC to look for signs of infection.

Treatment involves trying to minimize the symptoms. Providing adequate fluids and comforting foods can help soothe the sore throat. Rest is encouraged to help combat the infection. Pain and fever are treated with OTC medications that are recommended by the provider. If the infection is caused by a bacterium, the provider may prescribe an antibiotic. It is important to stress to patients the need to complete the full course of the antibiotics to ensure that the infection is fully resolved. A tonsillectomy, surgery to remove the tonsils, is performed when tonsillitis frequently recurs, with chronic tonsillitis, or when bacterial tonsillitis does not respond to antibiotic therapy.