Professional Documents
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FMS 3 Week 3
FMS 3 Week 3
2. Immune system
Immune system. Sistem imunitas suatu organisme
Immunity. Kemampuan sistem imunitas suatu organisme menjaga atau melindungi organisme tersebut dari
pathogen
Immune response. Response dari sistem immune ketika menghadapi pathogen.
Immune system components and interactions
4. Organs that are involved in immune system and components of immune system
Lymphoid organs
o The immune system is made up of organs that control the production and maturation of certain
defense cells, the lympocytes. Bone marrow and the thymus, a gland situated above the heart and
behind the breast bone, are so- called primary lymphoid organs. The bone marrow produces defense
cells. Some of these defense cells, the so- called T lymphocytes (T stands for thymus), are differentiated
in the thymus. That menas that this is where they develop into cells that are capable of recognizing non
self protein, so called antigens. The secondary lymphatic organs are the place where the defence cells
do their actual work. These organs include the lymph nodes, the spleen, the tonsils and other
specialized tissues in the mucous membranes of the bowel, for example. In these places, the defense
cells have constant contact with non- self substances and pathogens.
Bone marrow
o Bone marrow is a sponge like tissue situated inside of the bones. Most defense cells are produces and
then also multiply here. They then migrate from the bone marrow into the bloodstream and reach
other organs and tissues, where the defense cells mature and specialize. At birth, many bones contain
red bone marrow, which actively builds defense cells. During the course of life, more and more red
bone marrow turns into fat tissue. Adults only have red bone marrow in a few bonees, for example in
the ribs, in the breast bone and in the pelvic bone.
Thymus.
o The thymus, also called the thymus gland, is only fully developed in children. From adolescence
onwards, it is slowly turned into fat tissue. The gland like organ is situated behind the breast bone
above the heart. Certain defense cells are differentiated in the thymus, the so called T lymphocytes, or
T cells for short, among other things, are responsible for coordinating the innate and the adaptive
immune system. The T in T lymphocytes stands for the thymus, the place where they mature. T cells
move through the body and constantly watch the surface of all cells for changes. To be able to do this
job, they learn in the thymus which structures on cell surfaces are self and which are non self. When
coming into contact with a non self body, T cells turn into so called T effector cells, which trigger and
regulate different defense reaction. This type of cells includes T killer cells, which can destroy cells
infected in the pathogen. T helper cells are another kind of effector cells, which can destroy cells
infected with a pathogen. T helper cells are another kind of effector cells, which support other immune
cells in doing their work. In childhood, the thymus tissue also produced 2 hormones, thymosin and
thymopoietin, which regulate the maturation of defense cells in the lymph nodes.
Lymph nodes
o The lymphatic system of lymph nodes and vessels is important for continually ecchanging substances
between the blood and the tissue in the body. Fluid constantly leaves the blood, and defense cells and
protiens migrate into the surrounding tissue. Most of the fluid is later taken back into the blood
vessels. The rest of it is removed by the drainage system of the lymph vessels, which forms a fine net of
thin walled vessels in the body. The lymph nodes filter and clean the lymph fluids on its way to the
larger lymph vessels. The lymph finally travels into a vein called the superior vena cava, where it enters
the blood stream. Lymph nodes work like biological filter stations. They contain different defense cells,
which trap pathogens and activate the production of specific antibodies in the blood. If lymph nodes
become swollen, painful or hard, It can be a sign of an active defense reaction, for example in an
infection or, in rare cases, in malignant changes of the body’s own cell.
Spleen
o The spleen is situated in the left upper abdomen, beneath the diaphgram. It has a variety of tasks in the
defense system. In the unborn child, the spleen mainly produces blood and defense cells. After birth
this organ is mainly responsible for removing blood cells and for specific defence functions. As part of
the immune defense, the function of the spleen includes:
Stores different defense cells that are released into the blood to get to the organs, if needed:
macrophages, also called scavanger cells, can attack non self substances and pathogens
directly. T lymphocytes inspect cell surfaces, help in controlling defense and can also directly
destroy cells that have been recognize as non- self or as pathogens. B lymphocytes produce
antibodies, if needed.
It is resposible for removing red blood cells (erythrocytes).
Blood platelets (thrombocytes), which are responsible for blood clotting together, are stored
and removed in the spleen.
So there is always a lot of blood flowing through the spleen tissue. At the same time this tissue is very
soft. In heavy injuries, in an accident, for example, the spleen can therefore rupture easily. The spleen
then needs to be operated on, because otherwise there is a danger of bleeding to death. If the bleeding
cannot be stopped, and the spleen has to be removed, other defense organs take on most of its tasks.
Tonsils
o Tonsils also belong to the defense system. Due to their special position at the throat and palate, their
defense cells come into contact with pathogens especially soon, and can activate the immune system
immediately. Their tissue contains mainly lymphocytes. In addition to the palatine tonsils on the right
and left side, which are commonly just called tonsils, there are also the adenoids above the roof of the
throat, the lingual tonsil at the base of the tongue, and more lymphatic tissue on the sides of the
throat. This lymphatic tissue can take on the function of the adenoids, if these have been removed.
Lymphatic tissue in the bowel and other mucous membranes in the body
o The bowel plays a central role in defending the body against pathogens: more than half of all cells that
produce antibodies are found in the bowel wall, especially in the last part of the small bowel and in the
appendix. These cells recognize pathogens and other non self substances, and mark and destroy them.
They also store information of these non self substances to be able to react faster the next time. The
large bowel always contains bacteria that belong to the body, the so called gut flora. These bacteria in
the large bowel make it difficult for other pathogens to settle and to enter the body. The immune
system of the bowel tolerates the bacteria of the gut flora. Other parts of the body where pathogens
may enter also contain lymphatic tissue in the mucous membranes. All this tissue together Is also called
mucosa- associated lymphoid tissue (MALT). Pathogens might enter the body through the airways or
the urinary tract, for example. Lymphatic tissue can be found in the bronchi and in the mucous
membranes of the nose, the urinary bladder and the vagina with the defense cells being directly
beneath the mucous membrane where they prevent bacteria and viruses from attatching.
5. X linked agammaglobulinemia
Bruton type agammaglobulinemia, bruton syndrome, or sex linked agammaglobulinemia is a rare x linked geneti
disorder discovered in 1952 that affects the body’s ability to fight infection. XLA is an x linked disorder, and
therefore is much more common in males. XLA patients do not generate mature B cells, which manifests as a
complete lack of antibodies in their bloodstream. B cells are part of the immune system and normally
manufacture antibodies (called immunoglobulin), which defend the body from infections by sustaining an
immunological humoral antibody response. Patients with untreated XLA are prone to develop serious and even
fetal infections. A mutation occurs at the bruton’s tyrosine kinase gene that leads to a severe block in B cell
development (at the proB to preB cell stage) and a reduced immunoglobulin (antibody) production in the serum.
Btk is particulary responsible for mediating B cell development and maturation through a signaling effect on the
b cell receptor BCR. Patients typically present in early childhood with recurrent infections, in particular with
extravellular, encapsulated bacteria. It occurs in a frequenxy of about 1 in 100.000 male newborns, and has no
ethnic predisposition. XLA is treated by infusion of human antibody. Treatment with pooled gamma globulin
cannot restore a functional population of B cells, but it is sufficient to reduce the severity and number of
infections due to the passive immunity granted by the exogenous antibodies.
Treatment. The most common treatment for XLA is an intravenous infusion of immunoglobulin every 3-4 weeks,
for life. IVIG is a human product extracted and pooled from thousands of blood donations. IVIG does not cure
XLA but increases the patient’s lifesoan and quality of life, by generating passive immunity, and boosting the
immune system. With treatment, the number and severity of infections is reduced. With IVIG, XLA patients may
live a relatively healthy life. A patient should attempt reaching a stage where his igG blood count exceeds
800mg/kg. the dose is based on the patient’s weight and igG blood count. Muscle injections of immunoglobulin
were common before IVIG was prevalent, ut are less effective and much more painful; hence, Imig is now
uncommon. Subcuteneous treatment was recently approved by the US food and drug administration, which is
recommended in cases of severe adverse reactions to the IVIg treatment. Antibiotics are another common
supplementary treatment. Local antibiotic treatment (drops, lotion) are preferred over systemic treatment for
long term treatment, if possible. One of the future prospects of XLA treatment is gene therapy, which could
potentially cure XLA. Gene therapy technology is still in its infancy and may cause severe complications such as
cancer, even death. Moreover, the long term success and complications of his treatment.
Tonsils and lymph nodes in people with AXA. Tonsils and lymph nodes were supposed to be swollen in infection
scenario. Especially in babies, where the skin are still thin and relatively low connective tissue to body surface
area ratio makes it more visible. In people with AXA, lymph nodes and tonsils tend not to swell because the body
lacks B cell to produce. In infections scenario, B cell swell and releases antibodies. This “swelling” mechanism
causes an increase in the total surface area within the lymph vessel or the lymph node, causing a swelling. In AXA
patient, there has been a tremendous depletion of B cell production, resulting in this less “surface area increase”,
therefore lymph node was unobservable.
8. Perinatal
Periode yang muncul sekitar pada waktu kelahiran (5 bulan sebelumnya dan satu bulan sesudahnya). Periode
perinatal terjaid pada 22 minggu setelah periode gestasi lewat dan berakhir tujuh hari setelah kelahiran. Strategi
pemerintah dan inisiatif international mempromosikan menyusui sebagai metode terbaik pemberian makan
pada tahun pertama mereka.
9. Vaccine vs immunsation
Vaccination is when a vaccine is administered to you (usually by injection). Immunisation is what happends in
your body after you have the vaccination. The vaccine stimulates your immune system so that it can recognise
the disease and protect you from future infection (you become immune to the infections).
Vaccine are used to immunise people against infectious diseases. Immunsation helps prevent disease.
10. Immune system disorders
When your immune system doesn’t work the way it should, it is called an immune system or immunodeficiency
disorder. You may:
o Be born with weak immune system (primary immune deficiency)
o Get a disease that weakens your immune system (acquired immune deficiency)
o Have an immune system that is too active
o Have an immune system that turns against you (autoimmune disease occur)
Immunodeficiency disorders
o Severe combined immunodeficiency (SCID). This is an example of an immune deficiency that is present
at birth. Children are in constant danger of infections from bacteria, viruses, and fungi. This disorder is
sometimes called “bubble boy disease. In the 1970s, a boy had to live in a sterile environment inside a
plastic bubble. Children with SCID are missing important white blood cells.
o Temporary acquired immune deficiencies. Your immune system can be weakened by certain drugs, for
example. This can happen to people on chemotherapy or other drugs used to treat cancer, or to people
following organ transplant who take medication to prevent organ rejections. Also, infections like the flu
virus, mononucleosis, and measles can weaken the immune system for a brief time. Your immune
system can also be weakened by smoking, alcohol and poor nutrition.
o AIDS. HIV, which causes AIDS, is an acquired viral infection that destroys important white blood cells
and weakens the immune system. People with HIV/AIDS become seriuously ill with infections that most
people can fight off. These infections are called “oppurtunistic infections” because they take advantage
of weak immune systems.
An overactive immune system. If you are born with certain genes, your immune system may react substances in
the environment that are normally harmless. These substances are called allergies. Having an allergic reaction is
the most common example of an overactive immune system. Dust, mold, pollen, and foods are examples. Some
conditions caused by an overactive immune system are:
o Asthma. The response in your lungs can cause coughing, wheezing, and trouble breathing. Asthma can
be triggered by a common allergen like dust or pollen or by an irritant like tobacco smoke.
o Eczema. An allergen causes an itchy rash known as atopic dermatitis.
o Allergic rhinitis. Sneezing, a runny nose, sniffling, and sweeling of nasal passages from indoor allergens
like dust and pets or outdoor allergens like pollens or molds.
Autoimmune disease. In autoimmune disease, the body attacks normal, healthy tissues. The cause is unknown. It
is probably a combination of a person’s genes and something in the environment that triggers those genes. Three
common autoimmune disease are:
o Type 1 diabetes. The immune system attacks the cells in the pancreas that make insulin. Insulin
removes sugar from the blood to use as energy.
o Rheumatoid arthritis. It causes swelling and deformities of the joints. An auto- antibody called
theumatoid factors is in the blood of some people with rheumatoid arthritis.
o Lupus. Systemic lupus erythematosus is an autoimmune disease that attacks body tissues, including the
lungs, kidneys and skin. Many types of auto antibodies are found in the blood of people with lupus.
o Inflammatory bowel disease. The immune system attacks the lining of the intestines, causing episodes
of diarrhea, rectal bleeding, urgent bowel movements, abdominal pain, fever, and weight loss.
Ulcerative colitis and crohn’s disease are the two major forms of IBD. Oral and injected immune-
suppressing medicines can treat IBD.
o Multiple sclerosis. Immune system attacks nerve cells, causing symptoms that can include pain,
blindness, weakness, poor coordination and muscle spasms.
o Guillain-Barre syndrome. Immune system attacks the nerve controlling muscles in the legs and
sometimes the arms and upper body. Weakness results, which can sometimes be severe. Filtering the
blood with a procedure called plasmapheresis is the main treatment.
o Psoriasis. In psoriasis, overactive immune system blood cells called T cells collect in the skin. The
immune system activity stimulates skin cells to reproduce rapidly, producing silvery, scalpy plaques on
the skin.
o Grave’s disease. Immune system produces antibodies that stimulate the thyroid gland to release
excess amounts of thyroid hormone into the blodd.
o Hashimoto’s thyroiditis. Antibodies produced by the immune system attack the thyroid gland, slowly
destroying the cells that produce thyroid hormone. Low levels of thyroid hormone develop, usually
over months to years.
o Myasthenia gravis. Antibodies bind to nerve and make them unable to stimulate muscle properly.
Weakness that gets worse with activity is the main symptoms. Metinon is the main medicine used.
o Vasculitis. The immune system attacks and damages blood vessels in this group of autoimmune
diseases. Vasculitis can affect any organ, so symptoms vary widely and can occur almost anywhere in
the body.
11. B cells and T cell differences
Just like T cells, each B cells has a receptor that will connect to only one antigen shape. And like T cells, B cells
that recognize self- antigens are destroyed, so they don’t harm your body’s healthy cells. An important diffrence
between T cells and B cells is that B cells can connect to antigens right on the surface of the invading virus or
bacteria. This is different from T cells, which can only connect to virus antigens on the outside of infected cells.
Your body has up to 10 billion different B cells. They’re too small to see with your eyes, but if you lined them all
up, they’d be longer than 100 soccer fields. With so many different
B cells patrolling your body, you are ready to fight almost any
invader. When a B cell receptor connects to its specific antigen, a
helper T cell releases chemicals that tell that B cell to divide many
times. This makes an army of B cells with the perfectly shaped B cell
receptor to connect to the invader in your body.
Many of these B cells quickly turn into plasma cells. Plasma cells
make and release antibodies that connect to the same antigen as
the original B cell receptor. Plasma cells make thousands of
antibodies per second, which spread throughout your body,
trapping any viruses they see along the way.