Lymphatic System: Facts, Functions &

Diseases
By Kim Ann Zimmermann, Live Science Contributor | March 11, 2016 07:43pm ET
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Lymphocytes attack a cancer cell.

Credit: Juan Gaertner | Shutterstock

The lymphatic system is a network of tissues and organs that help rid the
body of toxins, waste and other unwanted materials. The primary function of

the lymphatic system is to transport lymph, a fluid containing infectionfighting white blood cells, throughout the body.
The lymphatic system primarily consists of lymphatic vessels, which are
similar to the circulatory system's veins and capillaries. The vessels are
connected to lymph nodes, where the lymph is filtered. The tonsils, adenoids,
spleen and thymus are all part of the lymphatic system.

Description of the lymphatic system

There are hundreds of lymph nodes in the human body. They are located
deep inside the body, such as around the lungs and heart, or closer to the
surface, such as under the arm or groin, according to the American Cancer
Society.
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The spleen, which is located on the left side of the body just above the
kidney, is the largest lymphatic organ, according to the U.S. National Library
of Medicine (NLM). It controls the amount of red blood cells and blood
storage in the body, and helps to fight infection. If the spleen detects
potentially dangerous bacteria, viruses, or other microorganisms in the
blood, it along with the lymph nodes creates white blood cells called
lymphocytes, which act as defenders against invaders. The lymphocytes
produce antibodies to kill the foreign microorganisms and stop infections
from spreading. Humans can live without a spleen, although people who
have lost their spleen to disease or injury are more prone to infections.

The lymphatic system helps keep the body healthy by eliminating infections
and diseases.
Credit: by Ross Toro, Infographics Artist

The thymus is located in the chest just above the heart, according to Merck
Manual. This small organ stores immature lymphocytes (specialized white
blood cells) and prepares them to become active T cells, which help destroy
infected or cancerous cells.
Tonsils are large clusters of lymphatic cells found in the pharynx. According
to the American Academy of Otolaryngology, they are the body's "first line of
defense as part of the immune system. They sample bacteria and viruses
that enter the body through the mouth or nose." They sometimes become
infected, and although tonsillectomies occur much less frequently today then
they did in the 1950s, it is still among the most common operations
performed and typically follows frequent throat infections.
Lymph is a clear and colorless fluid; the word "lymph" comes from the Latin
word lympha, which means "connected to water," according to the National
Lymphadema Network.
Plasma leaves the body's cells once it has delivered its nutrients and
removed debris. Most of this fluid returns to the venous circulation through
tiny blood vessels called venules and continues as venous blood. The
remainder becomes lymph, according to the Mayo Clinic.
Unlike blood, which flows throughout the body in a continue loop, lymph
flows in only one direction upward toward the neck. Lymphatic vessels
connect to two subclavian veins, which are located on either sides of the
neck near the collarbones, and the fluid re-enters the circulatory system,
according to the Mayo Clinic.

Diseases and disorders of the lymphatic system

Diseases and disorders of the lymphatic system are typically treated by
immunologists. Vascular surgeons, dermatologists, oncologists and
physiatrists also get involved in treatment of various lymphatic ailments.
There are also lymphedema therapists who specialize in the manual drainage
of the lymphatic system.
The most common diseases of the lymphatic system are enlargement of the
lymph nodes (also known as lymphadenopathy), swelling due to lymph
node blockage (also known as lymphedema) and cancers involving the
lymphatic system, according to Dr. James Hamrick, chief of medical oncology
and hematology at Kaiser Permanente in Atlanta.

When bacteria are recognized in the lymph fluid, the lymph nodes make
more infection-fighting white blood cells, which can cause swelling. The
swollen nodes can sometimes be felt in the neck, underarms and groin,
according to the NLM.
Lymphadenopathy is usually caused by infection, inflammation, or cancer.
Infections that cause lymphadenopathy include bacterial infections such as
strep throat, locally infected skin wounds, or viral infections such as
mononucleosis or HIV infection, Hamrick stated. The enlargement of the
lymph nodes may be localized to the area of infection, as in strep throat, or
more generalized as in HIV infection. In some areas of the body the enlarged
lymph nodes are palpable, while others are to deep to feel and can be seen
on CT scan or MRI.
Inflammatory or autoimmune conditions occur when a person's immune
system is active, and can result in enlargement of lymph nodes. This can
happen in lupus, according to Hamrick.
Lymphoma is cancer of the lymph nodes. It occurs when lymphocytes grow
and multiply uncontrollably. There are a number of different types of
lymphoma, according to Dr. Jeffrey P. Sharman, director of research
at Willamette Valley Cancer Institute and medical director of hematology
research for the U.S. Oncology Network.
The first branch point is the difference between Hodgkin lymphoma and
non-Hodgkin lymphoma (NHL), Sharman said. Non-Hodgkin lymphoma is
more common of the two, according to the Lymphoma Research Foundation.
The most common types of NHL are follicular, which accounts for about 30
percent of all NHL cases; diffuse large B-cell lymphoma (DLBCL), which
comprises 40 to 50 percent of NHL cases; and Burkitt's lymphoma, which
accounts for 5 percent of NHL cases. "The remainder of cases makes up the
bewildering complexity of NHL, Sharman said.
Though there can be a significant range within an individual category, the
clinical approach to each category is unique and the expectations of patient
outcome varies by category," Sharman said.
When a person has had surgery and/or radiation to remove a cancer, the
lymphatic flow back to the heart and can result in swelling or lymphedema,
Hamrick noted. This most commonly occurs in women who have had surgery
to remove a breast cancer. Part of the operation to remove the breast cancer
involves removing lymph nodes in the armpit.
The more lymph nodes removed the higher the risk of chronic bothersome
swelling and pain due to lymphedema in the arm, Hamrick explained.
Fortunately modern surgical techniques are allowing for fewer lymph nodes

to be removed, and thus fewer cases of severe lymphedema for breast

cancer survivors.
Castleman disease is a group of inflammatory disorders that cause lymph
node enlargement and can result in multiple-organ dysfunction, according to
the Castleman Disease Cooperative Network. While not specifically a cancer,
it is a similar to a lymphoma and is often treated with chemotherapy. It can
be unicentric (one lymph node) or multicentric, involving multiple lymph
nodes.
Lymphangiomatosis is a disease involving multiple cysts or lesions formed
from lymphatic vessels, according to the Lymphangiomatosis & Gorham's
Disease Alliance. It is thought to be the result of a genetic mutation.

Diagnosis and treatment

Diseases of the lymphatic system are usually diagnosed when lymph nodes
are enlarged, Hamrick noted. This may be discovered when the lymph nodes
become enlarged enough to be felt ("palpable lymphadenopathy") or are
seen on imaging studies such as CT scans or MRIs.
The majority of enlarged lymph nodes are not dangerous; they are the body's
way of fighting off an infection, such as a viral upper respiratory infection. If
the lymph nodes become significantly enlarged and persist longer than the
infection then they are more worrisome. There is no specific size cutoff, but
typically nodes that persist at larger than a centimeter are more worrisome
and warrant examination by a doctor.
Common symptoms of any lymphatic disorder include swelling of the arm or
groin, weight loss, fever and night sweats, according to Stephanie Bernik,
chief of surgical oncology at Lenox Hill Hospital in New York. A PET or CAT
scan is usually ordered to further investigate.
The diagnosis of lymphadenopathy depends on the location of the abnormal
lymph nodes and other things that are going on with the patient. If the
patient has a known infection, then the lymph nodes can simply be followed
to await resolution with treatment of the infection. If the nodes are growing
quickly and there is no obvious explanation then typically a biopsy is
warranted to look for a cancer or an infection. If the node can be felt then
this can be done at the bedside with a needle, according to Hamrick.
If the lymph node is deeper, such as in the abdomen or pelvis, Hamrick said
the biopsy might need to be done by an interventional radiologist using
image guidance to place the needle into the node. Sometimes the biopsy
needs to be done by a surgeon in the operating room. This is often where the
most tissue can be obtained to make a diagnosis, he said.

With many types of lymphoma and leukemia, there are unique treatment
options for each type, according to Sharman. There is no one summary of
treatment options. Treatment options can include traditional chemotherapy,
immunotherapy (such as using antibodies or immune modulating drugs), and
even radiation.
Treatment of lymphatic diseases depends on treating the underlying cause.
Infections are treated with antibiotics, supportive care (while the immune
system does its job, as in a viral infection) or antivirals. Lymphedema can be
treated by elevation, compression and physical therapy. Cancers of the
lymphatic system are treated by chemotherapy, radiotherapy, surgery, or a
combination of those modalities, Hamrick noted.
In last several years, Sharman noted that there has been explosion of new
treatment options. There are a handful of newly approved drugs that target
the actual disease causing processes within cells. Ibrutinib, idelalisib,
obinutuzumab, lenalidomide have been approved in various indications and
it is likely that we will see multiple more in coming year.