Thrombotic Thrombocytopenic Purpura, TTP, is a very rare autoimmune disease, only affecting 1 in every 100,000 people.

An autoimmune disease is when the immune system makes antibodies that attack the bodys own cells and tissues. A person who has TTP is missing a very important enzyme called the ADAMTS 13 enzyme. The ADAMTS 13 enzyme cut the ADAMTS 13 protein, which is about the size of a grape, into tiny pieces, so the protein can travel through the vascular systems primarily in the brain, heart, and kidneys. The reason that TTP patients are missing this enzyme is because their B-anitbodies attack the enzyme. Anti-bodies are proteins in the blood plasma that ght against foreign substances. When the enzyme is missing the grape sized protein gets clogged in the veins. When the protein gets clogged the body sends out signals to platelets that their is an injury. A platelets job is to stop blood clotting in an injury. Therefore when the platelets get sent to this injury they clog up the veins even more. When the veins are clogged nothing can get through, including things like red blood cells, whos job is to carry oxygen to other cells in the body. When the red blood cell reaches this clot in the vein and cant get through it tries so hard to squeeze through that it bursts open. This is called a shistocyte and is almost always present in TTP patients. Considering these things, someone with TTP will have a low red blood cell count and a low platelet count. Technically there were platelets there, they got used for the wrong reason. Their red blood cell count will be 5-7, 8-15 is normal, and their platelet count will be low 10,000, 150,00- 350,00 is normal. Although doctors are not sure of everything that can cause TTP, somethings they know can or might include;

! Thrombotic Thombocytopenic Purpura! !

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infections, live vaccines, underlying autoimmune conditions, abscessed teeth, ingrown toenails, certain medicines, and certain medical procedures. Luckily TTP is not passed from generation to generation. TTP commonly occurs after upper respiratory or bladder/ kidney infections. Pregnant women, who already have a weakened immune system from the foreign body inside them, can be more susceptible to TTP. It has been noticed that many TTP patients were taking Seasonale birth control when they were diagnosed with TTP, but no research has been done on this and doctors say that they are not connected. Symptoms of TTP can be very extreme and severe. Symptoms include; low platelet count, low red blood cell count, fatigue, fever, migraine headache, back pain, chest pain, bleeding (from nose and gums), diarrhea, kidney failure, dark urine, bruising, petechiae, bruising, change in level on consciousness, confusion, hallucinations, and an impending sense of doom. Everyone the has TTP will have some of these symptoms, but they may not have all of them. When treated correctly and immediately almost no one with TTP dies from it now, but TTP is denitely not one of the most known diseases and it has a very specic way that it must be treated. This makes diagnosing TTP at smaller hospitals a problem, because a low platelet and red blood cell count would almost immediately be treated by giving the patient units of RBCs and platelets. Obviously this would just add to the problem of blood clotting and would cause IMMEDIATE death. Although, when smaller hospitals do diagnose TTP correctly, by doing an ADAMTS 13 test, they have to send them to bigger hospitals because they do not have the correct equipment for treating TTP. Plasmapheresis, plasma exchange (PLEX), is the rst line of treatment that a TTP patient will receive. They will receive this along with a series of drugs that will help the immune system

function properly again. During PLEX the patient is connected to a large machine that removes blood from them. It then spins the blood in a centrifuge, a machine with a rapidly rotating container that applies centrifugal force to the contents (in this case the blood), it does this to separate uids of different densities. In this case the centrifuge removes the plasma from the blood, but returns the other components (red blood cells, white blood cells, and platelets), mixed with new and healthy plasma from a donor. The plasma must be replaced because it makes up 55 percent of the blood and contains red blood cells, platelets, and enzymes (including the ADAMTS 13). An autoimmune disease never truly goes away. If you have one you can go into a state of remission, but you can relapse anytime the immune system is compromised. Therefore, about 7 years ago it was decided to use Rituximab (Rituxan), a newer type of treatment called monoclonal antibodies, on TTP patients that are showing signs of relapsing. These signs are mainly low platelet, red blood cell, and ADAMTS 13 enzyme count. The normal immune system has antibodies to ght bacteria, fungi, and viruses. The anti bodies have names such as: IgG, IgA, IgM, B cells, T cells, etc. In TTP the B cells are directly related to the destruction of the ADAMTS 13 enzyme. Rituxan targets strictly the B antibody and lowers it. This lets the enzyme rebuild and allows a state of remission of return. Rituxan is given through an IV dose with different doses for each patient. The normal dose is usually 1000mg two times, fourteen days apart. Although some patients require more and some require less. It is a miracle that they are able to perform something and keep so many people from relapsing when just 7 years ago there was relapse, after relapse, after relapse. TTP relates very much to biology. It is caused by

the lack of an enzyme that is destroyed by B-antibodies, which leads to the build up of platelets, and the bursting of red blood cells. !