Thrombotic thrombocytopenic purpura (TTP) is a blood disorder where blood forms clots throughout the body in small blood vessels. The clots that form in body use up resources, like platelets, present in the blood. TTP occurs when some mechanism causes impaired activity of ADAMTS13, an enzyme responsible for the breakdown of a protein referred to as von Willebrand factor, which forms clots by binding with platelet clumps.
There are two different types of thrombotic thrombocytopenic purpura. Inherited TTP refers to a genetic mutation inherited from an individual's parents that impair the effect of ADAMTS13. Acquired TTP describes when the body produces proteins or antibodies that hinder the activity of ADAMTS13. Thrombotic thrombocytopenic purpura is diagnosed through numerous blood tests that reveal specific antibodies and activity levels of ADAMTS13. Treatment involves the use of steroid medications and plasma-like substances to replace the missing enzyme.
Shortness Of Breath
An individual may have thrombotic thrombocytopenic purpura if they experience shortness of breath frequently. The primary mechanism in thrombotic thrombocytopenic purpura that results in shortness of breath is a condition referred to as hemolytic anemia. Hemolytic anemia is a condition where an affected individual's red blood cells are being killed off at a faster rate than they are being produced. Red blood cells are responsible for transporting oxygen throughout the body. When there are not enough red blood cells in the body, the tissues around the body do not receive enough oxygen to operate properly.
The patient's brain can sense when tissues around the body are not receiving enough oxygen by detecting high levels of carbon dioxide. The brain reacts by sending signals to the heart and the lungs to increase the patient's breathing rate and heart rate. This mechanism is activated in an attempt to compensate for the low oxygen levels in the body. A faster breathing rate helps bring in large quantities of oxygen, and a faster heart rate helps move it around the body quicker. However, these mechanisms cause unpleasant symptoms in the patient, including shortness of breath.
Thrombotic thrombocytopenic purpura patients may develop a type of bruise or pinpoint rash referred to as petechiae. This kind of rash is best described as non-raised patches of tiny circular dots that manifest in an individual's serous or mucous membrane. These dots may be red, brown, or purple, and will not blanch or turn white when they are pressed on. Petechiae occurs when bleeding occurs underneath a patient's skin. Capillaries underneath an affected individual's skin break open and cause this type of bleeding.
The chances of spontaneous bleeding like the bleeding that causes a petechial rash are more significant in thrombotic thrombocytopenic purpura patients as a result of abnormally low platelets in the blood. Platelets are the tiny cells contained in the blood that fuse together and form clots when damaged blood vessels are discovered. Platelets keep an individual from losing an excessive amount of blood from bleeding too long. The petechial rash occurs because minor injuries to the individual's capillaries are not sealed fast enough due to platelet deficiency.
Easy bruising can occur in thrombotic thrombocytopenic purpura patients. A bruise is an injury where the skin becomes discolored due to an accumulation of damaged blood cells that congregates near the surface of an individual's skin. A bruise is usually blue, black, purple, brown, or yellow. Bruises in healthy individuals occur when they bump into something. The impact causes blood vessels to rupture or become injured, resulting in the accumulation of blood underneath the skin.
Bruises in healthy individuals are not problematic other than being mildly tender for several days because the body can mediate the blood vessel damage and bleeding with specific components in the blood. Platelets are responsible for sticking together to form a blood clot over an injury to stop the injured vessel from bleeding. Individuals who have thrombotic thrombocytopenic purpura, however, have low platelets in, which causes bruising to be more severe and more frequent upon minimal or no impact.
Jaundice Or A Pale Complexion
Individuals affected by thrombotic thrombocytopenic purpura develop a form of anemia where their red blood cells are being destroyed at a quicker rate than they are being produced. This form of anemia can cause the patient to have a pale complexion, due to a lack of proper oxygen delivery to their tissues. Jaundice is a yellow discoloration of the skin and whites of the eyes that may also manifest in thrombotic thrombocytopenic purpura.
Jaundice occurs when there is an elevated amount of a waste product referred to as bilirubin in the blood. Bilirubin is the result of the breakdown of hemoglobin, the substance inside of the red blood cells that allows them to transport oxygen. A healthy individual's liver can remove normal levels of bilirubin from the blood before they become an issue. However, an excessive amount of bilirubin accumulates in the blood of thrombotic thrombocytopenic purpura patients because the liver cannot clear higher quantities as effectively.
Fatigue can be caused by several factors but is most commonly associated with hemolytic anemia in thrombotic thrombocytopenic purpura patients. Hemolytic anemia is when an individual's red blood cells are being destroyed or killed faster than the body is producing new red blood cells, leading to a gross shortage of functional red blood cells. When there are not enough red blood cells, the tissues around the body do not receive enough oxygen to carry out their functional processes.
One critical process that requires oxygen in the cells is the generation of usable energy for the cell, a process referred to as ATP. When there is a shortage of energy being made around the body, the energy and oxygen are allocated to the cells and tissues considered critical. This mechanism leaves less overall oxygen and energy for the cells responsible for moving the muscles of the limbs because they are not as critical to survival as the cells that make up the vital organs. This shortage of energy makes a thrombotic thrombocytopenic purpura patient experience fatigue.