Lymphatic System

Functions of the lymphatic system

The key functions of the lymphatic system:

 Drains excess fluids and proteins from tissues all around the body and returns them back into
the bloodstream.
 Removes waste products produced by cells.
 Fights infections.
 Absorbs fats and fat-soluble vitamins from the digestive system and transports these into the
bloodstream.

Components of the lymphatic system

Lymph
Lymph is a fluid that circulates throughout the
body in the lymphatic system. It forms when
tissue fluids/blood plasma (mostly water, with
proteins and other dissolved substances) drain
into the lymphatic system. It contains a high
number of lymphocytes (white cells that fight
infection). Lymph that forms in the digestive
system called chyle, this contains higher levels
of fats, and looks milky white.
Lymph vessels
Walled, valved structures that carry lymph
around the body
Lymph nodes
Small bean-shaped glands that
produce lymphocytes, filter harmful substances
from the tissues, and contain macrophages,
which are cells that digest cellular debris,
pathogens and other foreign substances. Major
groups of lymph nodes are located in the
tonsils, adenoids, armpits, neck, groin and
mediastinum.
Thymus
The thymus is a specialized organ of the
immune system, located between the breast
bone and heart. It produces lymphocytes, is
important for T cell maturation (T for thymus-
derived).
Spleen
The spleen is an organ in the upper left
abdomen, which filters blood, disposes of worn-
out red blood cells, and provides a 'reserve
supply' of blood. It contains both red tissue, and
Immage source: Wikimedia License: CC- white lymphatic tissue. Different parts of the the
BY-3.0 spleen specialize in different kinds of immune
cells.

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The major (encapsulated) lymphatic organs are the lymph nodes, thymus and spleen. In addition the
lymphoid tissues include:

Mucosa-associated lymphoid tissue (MALT)

These are bundles of lymphatic cells, called lymphatic nodules, located within the mucus
membranes that line the gastrointestinal, respiratory, reproductive, and urinary tracts. These
nodules contain lymphocytes and macrophages which defend against invading bacteria and
other pathogens that enter these passages along with food, air, or urine. These nodules can
be solitary or grouped together in clusters.

Major clusters of lymphatic nodules include:

 Tonsils: these are clusters of lymphatic tissue under the mucous membrane lining of
the nose, mouth, and throat. Lymphocytes and macrophages in the tonsils provide
protection against foreign substances and pathogens that enter the body through the
nose or mouth.
 Adenoids: A cluster of lymphatic tissue that hangs from the upper part of the back of
the nasal cavity. Adenoids get bigger after birth but usually stop growing by the age of
7. Like the Tonsils, they can be removed without significantly increased risk of
infections.
 Peyer's patches: these are clusters of lymphatic nodules in the mucosa that lines the
ileum of the small intestine. They play an important role in defending against the large
number of pathogens that enter the gastrointestinal system.

Circulation of tissue fluids

Fluid in the spaces between tissues is called interstitial fluid, or 'tissue fluid'. This provides the cells
of the body with nutrients (via the blood supply) and a means of waste removal. Lymph is formed

when the interstitial fluid is collected through tiny lymph capillaries (see diagram), which are located
throughout the body. It is then transported through lymph vessels to lymph nodes, which clean and
filter it. Lymph then flows on to the lymphatic ducts, before emptying into the right or the
left subclavian vein, where it mixes back with blood.

Blood is enriched with oxygen (by the respiratory system) and nutrients (by the digestive system),
which are circulated all around the body (by the cardiovascular system). Some fluid (blood plasma)

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leaks out into the tissues via tiny capillaries, contributing to interstitial fluid, which eventually drains
back into the lymphatic system.

The Immune System

The immune system includes a variety of defenses against viruses, bacteria, fungal infections, and
parasites (such as thread worms). The lympatic system is part of the broader Immune System.

Innate immune system

This are the non-specific, unchanging lines of defenses which include:

 Physical and chemical barriers to pathogens.

 Producing cytokines and other chemical factors to recruit immune cells to sites of
infection.
 Activates the complement cascade to identify bacteria, activate cells and to promote
clearance of dead cells or antibody complexes.
 Identifies and removes foreign substances present in organs, tissues, the blood and
lymph, by specialised white blood cells.
 Activation of the adaptive immune system, through a process known as antigen
presentation.

Adaptive immune system

Adaptive (or acquired) immunity is where immunological memory is made after an initial
response to a new pathogen, leading to an enhanced response to future exposure to that
same pathogen. This process of acquired immunity is the basis of vaccination. This is
essential because bacteria and viruses are continually adapting and evolving in an 'arms race'
with our immune systems. Features of the adaptive immune system include:

 Recognition of specific "non-self" antigens, during the process of antigen

presentation.
 The generation of responses tailored to destroy specific pathogens or pathogen-
infected cells.
 Development of immunological memory, in which each pathogen is "remembered" by
signature antibodies or T cell receptors. These memory cells can be called upon to
quickly eliminate a pathogen should subsequent infections occur.

Cells of the Immune System

There are many different cell types and sub-types involved in the immune system. Some of
the main types include:

 Lymphocytes: are white cells which circulate between blood and lymph. They play
an important role in fighting infection. There are many kinds of lymphocytes; the main
types are T cells, B cells and natural killer cells. Lymphocytes initially develop in
the bone marrow. Some migrate to the thymus, where they mature into T cells ;
others mature in the bone marrow as B cells.
 Neutrophils: are the most abundant type of white blood cells and are an important
part of the innate immune system. Neutrophils are a type of phagocyte (cells which
engulf and then digest, cellular debris and pathogens). They are normally found in the
blood stream, but are quickly recruited to the site of injury or infection following
chemical signals such as Interleukin-8.
 Macrophages: are another type of phagocyte and have a role in both the innate and
adaptive immune systems. They attack foreign substances, infectious microbes and
cancer cells. Macrophages also stimulate lymphocytes and other immune cells to
respond to pathogens.

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  Dendritic cells: are antigen-presenting cells which act as messengers between the
innate and adaptive immune systems. They are usually located in tissues in contact
with the external environment such as the skin, linings of the nose, lungs, stomach
and intestines. In response to pathogens they migrate to the lymph nodes where they
interact with T cells and B cells to initiate the adaptive immune response.

Antigens and Antibodies

Antibodies (also known as an immunoglobulins) are Y-shaped proteins produced by B-
cells,that bind to specific antigens on the surface of foreign objects such as bacteria and
viruses. This identifies and 'tags' the foreign object as 'non-self', signalling other immune cells
to attack them.
Hormones and the Immune System
There are several hormones generated by the immune system. These hormones are
generally known as lymphokines. Steroids and corticosteroids (components of adrenaline)
suppress the immune system.

Cancer Focus
Metastatic spread of cancer via the lymph nodes
Lymph nodes close to the primary tumor are often the first site of metastases (spread of
cancer). Lymph node metastases are rarely life threatening, but their detection is a prognostic
factor for many types of cancer as it shows the tumor has developed the ability to spread.
Tumor cells may travel via the lymphatic system and spread to to lymph nodes and distant
organs.
Sentinel Lymph Node Biopsy
A dye is injected near the primary tumor to identify the position of the sentinel lymph node
(the first lymph node to which cancer cells are most likely to spread as the lympatic system
drains fluid away from the tumor). The sentinel node is surgically removed and a pathologist
checks for the presence of cancer cells. SLNB is most frequently used to help stage breast
cancer and melanoma. It is a less extensive operation compared to standard lymph node
surgery.
Immunosuppression
This is reduced activity or efficiency of the immune system and its ability to fight infections
and other diseases. Certain diseases such as AIDS or lymphoma can cause
immunosuppression. It is also a common side-effect of anticancer chemotherapy, leading to
cancer patients having an increased risk of infections during treatment.
Lymphoma
A general term form for malignant disease of the lymphatic tissue characterized by abnormal,
uncontrolled cell growth. There are a number of types of lymphoma, including Hodgkin
Lymphoma, with most other types classed together as Non-Hodgkin Lymphoma.
Hodgkin Lymphoma
A malignancy of the lymphatic tissue that occurs most often in males, and the peak incidence
is between ages 15 and 35. It is characterised by progressive, painless enlargement of the
lymph nodes, spleen, and general lymph tissue. In Hodgkin Lymphoma Reed-Sternberg cells
(a specific type of lymphocyte) become abnormal and grow in an uncontrolled way.
Internet Resources for Hodgkin Lymphoma
Non Hodgkin Lymphoma (NHL)
NHL is cancer of the lymphatic tissue, that does not involve abnormal Reed-Sternberg cells (a
specific type of lymphocyte). There are many different types of NHL. Some grow very slowly,
whilst others grow quickly and need aggressive treatment.
Internet Resources for NHL
AIDS related lymphoma
Incidence of non-Hodgkin's lymphoma has increased in parallel with the AIDS epidemic.
Lymphomas affecting HIV infected people are mostly of the aggressive B-cell types (diffuse
large cell, B-immunoblastic, or small non-cleaved Burkitt's / Burkitt's like lymphoma) which are

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 less common in non-HIV infected lymphoma patients. The HIV virus is not thought to a direct
cause of lymphoma, rather it weakens the body's defences and may increase susceptibility to
other infections such as the Epstein-Barr and HHV-8 viruses which are associated with these
types of lymphomas.
Internet Resources for AIDS related Lymphoma
Waldenstrom's Macroglobulinemia
This is a rare malignant condition, involving an excess of beta-lymphocytes (a type of cell in
the immune system) which secrete immunoglobulins (a type of antibody). WM usually occurs
in people over sixty, but has been detected in younger adults.
Internet Resources for Waldenstrom's Macroglobulinemia
Cancer Immunotherapy
This is treatment to stimulate the patient's own immune system to attack the cancer cells.
Different approaches include: 1) cancer vaccination to train the immune system to recognise
the cancer cells as targets to be destroyed, 2) giving therapeutic antibodies to recruit
immune system cells to destroy tumor cells, and 3) cell based immunotherapy which is
either transfusing immune cells (such as Natural killer Cells) or by administering cytokines
(such as Interleukins) which activate the immune cells.
HPV Vaccination and Cervical Cancer
Human papillomavirus (HPV) is a common cause of infection. There are over 100 different
sub-types of HPV. HPV types 16 and 18 cause 70% of cervical cancers and are also linked to
cancers of the anus, vulva, vagina, penis, as well as the mouth and throat. Over time these
can cause cells in the cervix to change, leading to precancerous conditions - cervical
intraepithelial neoplasia (CIN), with a higher risk of developing cancer. Vaccination against
HPV 16, 18 and other 'high risk' types of HPV reduces the risk of developing cervical and
other HPV-related cancers.
Internet Resources for HPV Vaccination and Cervical Cancer
Lymphedema
Lymphedma is an abnormal build up of interstitial fluid due to problems in the lymphatic
system. It can have many causes. In the context of cancer it is often a result of obstruction by
a tumor or enlarged lymph nodes. It can also be a side effect of radiotherapy or surgery,
which has damaged the lymph vessels.

Roots, suffixes, and prefixes

Most medical terms are comprised of a root word plus a suffix (word ending) and/or a prefix
(beginning of the word). Here are some examples related to the Lymphatic and Immune systems. For
more details see Chapter 4: Understanding the Components of Medical Terminology

component meaning example

aden(o)- gland Lymphadenopathy - disease of, or swelling/enlarged lymph nodes

Immunosuppression = reduced activation or efficacy of the immune

immun(o)- Immunity
system

lymph(o)- Lymph Lymphoma = tumour of lymphoid cells

lymphaden(o)- lymph node Lymphadenectomy = surgical removal of lymph node(s)

lymphatic
lymphangi(o)- Lymphangitis = inflammation or infection of the lymphatic vessels
vessels

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 splen(o)- spleen Splenomegaly = enlargement of the spleen

thym(o)- thymus Thymectomy = surgical removal of the thymus

Immunotoxicity = adverse effects on immune system function

tox(o)- poison
resulting from exposure to chemical substances.

Related Abbreviations and Acronyms

AIDS Acquired Immunodeficiency Syndrome

EBV Epstein-Barr virus

HD Hodgkin's Disease (now known as Hodgkin Lymphoma)

HIV Human Immunodeficiency Virus

HPV Humapapillomavirus

HSV Herpes Simplex Virus

IgA Immunoglobulin A

IgD Immunoglobulin D

IgE Immunoglobulin E

IgG Immunoglobulin G

IgM Immunoglobulin M

MALT Mucosa-associated lymphoid tissue

NHL Non Hodgkin's Lymphoma

NKT Natural killer T cell

SLNB Sentinel Lymph Node Biopsy

DISEASES OF LYMPHATIC SYSTEM

The malfunctioning or damage to any part of the lymph circulation mechanism, usually, results
in severe and acute lymphatic system diseases. Nevertheless, proper care of your health and
timely diagnosis of the abnormality can save you from a lot of future complexities. Some of the
commonly occurring disorders include lymphatic filariasis, elephantiasis, lymphangiosarcoma,
lymphangiomatosis, castleman's disease, Hodgkin's lymphoma, lymphedema, lymphoid
leukemias & lymphomas, lymphangioleiomyomatosis, and so on. Here follows brief descriptions
of such disorders with symptoms and treatment measures:

Lymphatic Filariasis

Also spelled as philariasis, this parasitic disease derives its name from the super family of the
nematodes (filarioidae or filariae) which are its causative agents. The black flies, mosquitoes
and other blood-feeding arthropods serve as carriers of the disease and transmit it from one
individual to another. Over one hundred and twenty million (120,000,000) individuals worldwide
show the symptoms of the disease, such as, the thickening of the skin and underlying tissues. It
is diagnosed with the help of Finger Prick Test, while the treatment may involve the
medications, like albendazole.

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Elephantiasis

Caused by the microscopic, thread-like parasitic worms, elephantiasis affects different organs
of the body, especially the legs and male genitals. As the very name suggests, it is
characterized by the thickening, and sometimes extreme swelling, of the skin and underlying
tissues. Owing to the awareness campaigns, launched internationally, nearly 7 million children
have been saved from the possible attack of the disease, and in about 10 million individuals, its
progress has been stopped. Albendazole is used in combination with either ivermectin or
diethylcarbamazine for the treatment of this infectious disorder transmitted by the mosquito
bites.

Lymphangiosarcoma

Occurring in the long standing cases of primary or secondary lymphedema, it is a rare disorder
that is characterized by the growth of a malignant tumor. However, the initial signs involve the
appearance of a purplish discoloration or a bruise mark, and gradually progresses to an ulcer
with crusting and, finally, results in extensive necrosis. The treatment usually depends on the
type and severity of the malignancy. In case of metastasis, chemotherapy may be
administered, and in some other cases, amputation of the affected limb is considered to be the
most successful therapy.

Lymphangiomatosis

The congenital errors of the lymphatic development occu rring before the 20th week of gestation
lead to this lymphatic malformation. It is marked by the formation of cysts which appear when
the abnormally interconnected and dilated thin-walled vessels increase both in size as well as
number. Mostly involving bones, it may also affect lungs and other organs or organs systems of
your body. As the disorder is very likely to attack more than one organ systems, it is usually
termed as a multi-system disease. The symptoms start appearing in the advanced stages of the
disease. Diagnosis can be carried out with plain X-rays which may reveal the presence of lytic
lesions in bones and interstitial infiltrates in the lungs. Depending on the severity of the
condition, different treatment approaches are recommended including medications and surgical
interventions.

Castleman's Disease

This rare disorder of the lymphatic system may affect either a single lymph node or spread
systematically to other nodes as well. The former condition is termed as unicentric while, in the
latter case, it is referred to as multicentric. To make distinction between the castleman’s
disease and reactive lymph node hyperplasia, the former is also named as angiofollicular lymph
node hyperplasia. The unicentric form of the disorder can often be cured w ith surgical
resection, but for multicentric condition there is no standard therapy.

Hodgkin's Lymphoma

Commonly known as Hodgkin's disease, it is characterized by the painless, gradual swelling of

the lymph nodes, persistent fatigue, and weight loss. In m ost of the cases, this rare medical
condition affects lymphatic tissues in the lymph nodes and spleen. It also shows the
characteristics of cancer as the infected node shows rapid and abnormal increase in size. If
such a cancerous condition remains unchecked, the malignancy will progress from the nodes
and inflict other organs as well, such as, liver, lungs and bone marrow. The successful
treatment is carried out either through the process of 'staging', or it may involve the application
of chemotherapy, radiations and autologous bone marrow transplantation.
A type of cancer of the lymphatic system.
Symptoms: Lymphadenopathy, Night Sweats, Anorexia, Fatigue, Pruritus, + more

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Treatments: Bleomycin, COPP-ABVD Regimen, Radiation therapy, Sargramostim, Procarbazine, +
more
Risk Factors: Family History of Hodgkin's Disease, Immunosuppression, Younger age, Prolonged
use of human growth hormone, Male, + more
Parent Disease: Cancer, Blood disorder, Lymphoma

Order of spread of Hodgkin's lymphoma

1. Local spread
2. Organs thru lymph
3. T lymphocytes defective & destroyed
3. Dec. # of T lymphocytes
What is the main diagnostic marker for Hodgkin's lymphoma?
Reed-Sternberg Cells-abnormally large

Stages of Hodgkin's lymphoma

I: Lymph involved (1 node)
II: 2 lymph involved (same side of diaphragm)
III: Node on both sides of diaphragm
IV: Diffuse extra-lymph involvement (organs, bones)
Hodgkin's lymphoma affects _ cells
T
Non-hodgkin's lymphoma affects _ cells
B

Lymphedema

Alternatively spelled as ‘lymphoedema’, this condition is caused by the compromised lymphatic

system. Lymphatic Obstruction (another name of the disease) occurs due to the localized fluid
retention and results in serious tissue swelling. That is why the pa tients are said to be at an
immediate risk of developing an infection. Some of the common symptoms include severe
fatigue, swollen limbs & other body areas, discoloration of the skin, and even deformity.
Depending upon the severity of the edema and degree of fibrosis of the affected limb, the
treatment measures vary. The most common treatments recommended by certified
lymphedema therapists include compression bandaging, compression garments or manual
compression lymphatic massage. Any of these treatments ca n be adopted either individually or
in combination with the other.

Lymphoid Leukemia

Also known as lymphocytic leukemia, it is a type of leukemia which specifically affects the
circulating lymphocyte cells. Here it is necessary to make distinction between lymphoma and
lymphoid leukemia, where the former refers to the formation of the solid mass of the same type
of cells. In the early stages, the symptoms usually do not appear, but later on, the patient has
to suffer from fatigue, fever, night sweats, weight loss, frequent infection and enlarged but
painless lymph nodes. Treatment is usually not necessary in the early stages, but the
intermediate and advanced stages may require cure through chemotherapy, targeted drug
therapy and bone marrow stem cell transplant.

Lymphangioleiomyomatosis (LAM)

This rare lung disease leads to the proliferation of the disorderly smooth muscle cells that may
spread to the entire lung region. As the small airways get obstructed, the lymphatics,
bronchioles, alveolar septa and perivascular spaces get badly disturbed. Usually affecting the
females of the child bearing age, the LAM occurs in sporadic form, and its chances increase if
the patient is already suffering from tuberous sclerosis. The prominent symptoms are: cough,
shortness of breath, recurrent pneumothorax and hemoptysis. The therapists have to choose

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from a number of available treatment measures, for example, anti -estrogen therapy, sirolimus
and lung transplantation

Odema
Treatments: Cyclothiazide, Indapamide, Hydroflumethiazide, Captopril, Ethacrynate Sodium, + more

Environmental Illness - Topic Overview

What is an environmental illness?

An environmental illness can occur when you are exposed to toxins or substances in the environment that
make you sick. These health hazards may be found where you live, work, or play.

Maybe you have headaches that only occur on weekends. Or maybe you began to feel sick and got a rash
after moving into a newly built home. These symptoms can be caused by exposure to toxic chemicals. For
example:

 Those weekend headaches may be caused by a broken furnace leaking carbon monoxide.
 Materials in new buildings may cause nausea and rashes. And the paper that makes up the
outside layers of drywall promotes mold growth. Exposure to these molds may cause symptoms
and could make you sick.

What causes environmental illnesses?

Exposure to some types of chemicals can cause an environmental illness. The more of the chemical you
are exposed to, the more likely you are to get ill. Examples include:

 Chemicals in cigarettes are known to cause lung cancer.

 Exposure to asbestos, an insulating material found in some older buildings, can cause
tumors, lung cancer, and other diseases.
 Wood-burning stoves and poorly vented gas ranges can produce smoke or gases that can
cause breathing problems.
 Unsafe drinking water from a rural well polluted with pesticides or other poisons from a nearby
industrial plant could cause allergies, cancer, or other problems.
 Certain chemicals in the workplace may cause sterility in men or fertility problems in women.
 Lead poisoning can cause health problems, most commonly in children. It can also cause high
blood pressure, brain damage, and stomach and kidney problems in adults.

What are the symptoms?

Symptoms of an environmental illness depend on what is causing it. The symptoms may be like those you
can get with other conditions. Examples are:

 Headache.
 Fever and chills.
 Nausea.
 A cough.
 Muscle aches.
 A rash.

If you think that exposure to toxic chemicals or other health hazards could be making you sick, talk to your
doctor.

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How are environmental illnesses diagnosed?

An environmental illness can be hard to diagnose. You and your doctor may not know what is causing
your symptoms. Or you may mistake your symptoms for another problem. Exposure to toxic chemicals can
cause a wide range of common medical problems or make them worse.

An exposure history, which is a set of questions about your home, workplace, habits, jobs, lifestyle, and
hobbies, can help you find out what is making you sick. It may point to chemicals or other hazards that
you've been exposed to recently or in the past.

Keep a journal of your symptoms, and discuss it with your doctor. It may help you find patterns in your
symptoms. This can help you and your doctor find out what is causing your illness.

How are they treated?

Early treatment includes stopping or reducing your exposure to what is making you sick. These things
might help:

 Improve your air quality by getting rid of the source of pollution.Don't allow smoking in your
house. If smokers live in or visit your home, ask them to smoke outside.
 Increase the amount of fresh air coming into your home. Adjust gas stoves, or replace them
with electric ones. Check to make sure that exhaust fans work. Installing carbon monoxide alarms
in your home can also protect you and your family.
 Stop the health effects of mold exposure. Keep a dry environment indoors to reduce exposure
to mold. If you do find mold, it should be removed. If the moldy area is less than 3 ft (1 m) by 3 ft (1
m), you can probably remove the mold yourself. But if the moldy area is bigger, a trained
professional should remove the mold.

Further treatment will depend on your symptoms and what is causing your illness.

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