Idiopathic thrombocytopenic purpura is an autoimmune deficiency of blood platelets. Patients suffer from spontaneous bleeding. The phenomenon is treated with medication, which leads to a cure in 70 percent of all cases.
What is Idiopathic Thrombocytopenic Purpura?
According to abbreviationfinder, Idiopathic thrombocytopenic purpura is determined using laboratory diagnostics against the background of anamnesis. In the anamnesis, past infections can provide a crucial clue.
Thrombocytopenia is a lack of blood platelets. There are normally around 150,000 to 450,000 platelets in one centilitre of blood. Falling below these guideline values is thrombocytopenia. The deficiency symptoms are caused either by reduced formation, disturbed blood platelet distribution or unnaturally high platelet degradation.
If the thrombocytopenia is below 80,000 per centilitre, a tendency to bleed is to be expected. Values below 50,000 per centilitre cause spontaneous nosebleeds and hematomas, cerebral hemorrhages and gastrointestinal bleeding. Idiopathic thrombocytopenic purpura (ITP) is also known as immune thrombocytopenia, haemorrhagic purpura, autoimmune thrombocytopenia, thrombocytopenic purpura or immune thrombocytopenic purpura.
This is an autoimmune disease that attacks the blood platelets. The subtypes are acute immune thrombocytopenia in children and chronic immune thrombocytopenia in adults. The latter form was first described by Werlhof and Wichmann and is better known as Werlhof’s disease, Werlhof-Wichmann syndrome or Werlhof’s disease.
This variant primarily affects women and occurs in around 100 people per million in a year. Disease is also called Evans syndrome against the background of autoimmune hemolytic anemia.
Causes
Idiopathic thrombocytopenic purpura results from a misdirected immune process. The cause of the platelet deficiency is therefore an autoimmune disease. The patient’s immune system forms free and platelet-bound antibodies against adhesion molecules such as Gp IIb/IIIa and thus shortens the lifespan of the blood platelets.
As with all other autoimmune diseases, the primary cause of idiopathic thrombocytopenic purpura has not yet been finally clarified. Various speculations are available as explanations. In the meantime, it has been observed that the disease is often preceded by infections of the respiratory tract or those of the gastrointestinal tract. Infections with HIV or EBV are often also present in the past.
Therefore, it is now assumed that cross-reactive antigens of the viral pathogens cause antibody formation within the framework of molecular mimicry, above all the formation of immunoglobulin G. Nevertheless, not all documented cases can be explained with infections.
For a long time it was not at all clear that the phenomenon was actually an autoimmune disease. Although the exact pathogenesis and cause of the disease has not yet been fully clarified, science now at least agrees on the autoimmunological basis.
Symptoms, Ailments & Signs
The symptoms of patients with idiopathic thrombocytopenic purpura differ from case to case. The disease is associated with an extremely variable clinical picture and can cause a number of symptoms in individual cases. The disease is always based on an increased breakdown of blood platelets. On this basis, the platelets decrease.
Since the blood platelets play an important role in the clotting cascade, the reduced number of thrombocytes results in a more or less strong tendency to bleed. Many patients spontaneously experience pinpoint bleeding from the skin, also known as petechiae. An external cause for the spontaneous bleeding cannot be determined.
In addition to the legs, the mucous membranes in the throat area are particularly affected, which gave the disease its name. In individual cases, spontaneous nosebleeds can also occur. Sick women also often suffer from prolonged menstrual bleeding. Since the disease can also cause cerebral hemorrhage or bleeding processes in the gastrointestinal tract, life-threatening conditions can result in extreme cases.
In the event of a cerebral hemorrhage, for example, the intracranial pressure increases. The brain is compressed in the skull and can suffer permanent damage. Normally, clinically manifest symptoms only occur when the platelet count is less than 30,000 per centilitre. Values of less than 10,000 platelets per centilitre cause life-threatening conditions. Nevertheless, life-threatening situations rarely occur in idiopathic thrombocytopenic purpura.
Diagnosis & course of disease
Idiopathic thrombocytopenic purpura is determined using laboratory diagnostics against the background of anamnesis. In the anamnesis, past infections can provide a crucial clue. The blood test shows a lack of platelets. Symptoms similar to the disease also cause thrombotic thrombocytopenic purpura, hemolytic uremic syndrome and heparin-induced thrombocytopenia.
These diseases must be excluded in the differential diagnosis. Ideally, the doctor will come across the presumably primary cause of infection in the course of diagnostics. The prognosis is relatively good with healing rates of around 70 percent. However, around four percent die of cerebral hemorrhage.
Complications
In many cases, this disease is diagnosed late because there are no uniform or characteristic symptoms and signs. For this reason, treatment unfortunately cannot be carried out early, which limits the prospects of a complete cure. As a rule, however, there is a breakdown of blood platelets.
As a result of this breakdown, those affected experience increased bleeding, which can occur in different regions of the body. In most cases, the bleeding occurs directly on the skin and there is spontaneous bleeding that is not associated with an injury. Furthermore, the patients suffer from frequent nosebleeds. In the worst case, it can lead to bleeding in the brain or stomach, which can lead to death if left untreated.
The bleeding in the brain increases the intracranial pressure, causing severe headaches and possibly paralysis. Treatment is usually with the help of medication and often leads to a positive course of the disease. However, it cannot be predicted whether a certain drug will lead to a cure in a patient or whether another one must be taken as well. Left untreated, life expectancy is reduced.
When should you go to the doctor?
If bleeding occurs repeatedly without an apparent reason, a doctor is needed. Sudden blood in the stool or urine that occurs over several days or intermittently is cause for concern. A doctor’s visit is necessary to clarify the cause. Frequent unexpected nose or gum bleeding are indicators of a discrepancy that should be investigated. Bleeding of the skin, changes in the appearance of the skin and spontaneous bleeding on the body are warning signs that must be investigated. If the bleeding is difficult to stop, a doctor should be informed of the observations.
If cerebral hemorrhage occurs, a life-threatening condition can develop. Therefore, a doctor’s visit is necessary as soon as dizziness, unsteady gait, sleep disorders or general malaise occur. If disturbances of consciousness occur, a doctor must be consulted immediately. In the event of a loss of consciousness, an emergency doctor must be calledbe called because an acute condition has occurred that requires immediate action. Until it arrives, first aid measures must be taken. Women should be examined if they suffer from heavy and at the same time prolonged menstrual bleeding. If the menstrual period lasts longer than 7 days, this is considered unusual and can be a signal for disorders in the organism. A doctor must be consulted in the event of heart palpitations, changes in blood pressure or irregular heart rhythms.
Treatment & Therapy
In some cases of ITP, no therapy is required because spontaneous healing occurs. If no spontaneous healing occurs, glucocorticoids are given. In addition, immunoglobulin therapy or intravenous administration of anti-D immunoglobulin is often carried out. In the case of severe bleeding complications, a splenectomy is performed since the immunological malfunction is usually to be expected in the spleen.
In addition, transfusions of foreign platelet concentrates can be carried out in the event of bleeding complications. In some cases, however, this leads to a worsening of the situation. Cytostatics suppress autoantibody-producing cells. Alternatively, monoclonal antibodies can be administered in combination with immunoglobulins. In addition, the administration of a non-structural analogous thrombopoietin preparation has been used since the recent past.
Each patient responds better to a different drug. These differences must be taken into account by carrying out regular checks. The treatment plan is usually changed several times in the therapy of an ITP before decisive successes can be seen. Some scientists assume that the 70 percent healed patients were cured of thrombocytopenia solely by self-healing. In fact, against the background of self-healing, it is difficult to assess the actual effectiveness of the individual drugs.
Outlook & Forecast
Idiopathic thrombocytopenic purpura – now known as immune thrombocytopenia (ITP) – usually has a good prognosis in adults. However, a prerequisite for healing is that an optimal course of therapy can be achieved. In addition, there must be no bleeding in the brain. In adults, idiopathic thrombocytopenic purpura is 70 to 80 percent curable. But it is also a fact that the prospects are worse when recurrences occur.
In children, idiopathic thrombocytopenic purpura takes a slightly different course. In younger children, idiopathic thrombocytopenic purpura is more likely to present as an acute disease. Immune thrombocytopenia often occurs after an infection. The chronic form of idiopathic thrombocytopenic purpura is rarely encountered in young children and adolescents. Therefore, the course of the disease is different. Overall, the prognosis is better than in adults. If only mild bleeding occurs, therapeutic intervention can be dispensed with. Spontaneous healing is even possible in children. A certain number of children and adolescents die as a result of intracerebral hemorrhage.
The mortality rate for idiopathic thrombocytopenic purpura is 4 percent of those affected. The prognosis is poor if cerebral hemorrhage occurs during the course of the disease. In recurrences, removal of the spleen sometimes improves ITP. However, recurrences can also occur after the operation.
Prevention
Idiopathic thrombocytopenic purpura is apparently a late consequence of infections. In this way, the phenomenon could be prevented at least to a limited extent by general infection prophylaxis.
Aftercare
Follow-up care for idiopathic thrombocytopenic purpura depends on the age of the patient and the individual course. If it is a late effect after an infection, people can take preventive measures to reduce the risk of a recurrence. With the same therapy, however, patients can react differently to the medication, which affects the healing process.
For this reason, those affected should be in close contact with their doctor and help with the selection of medication. The body’s self-healing powers also play a decisive role in the recovery process. They need medical support. It is therefore important that patients make an early appointment with their doctor if they notice any abnormalities.
During their stay in the hospital and also in the subsequent phase, patients should observe themselves and follow the medical orders. In addition, they can adjust their lifestyle to stabilize their health. With a nutritious diet and sufficient activity, they improve their fitness.
When it comes to patient self-responsibility, timely detection of possible complications is also an important issue. In this way, sudden bleeding can be detected at an early stage. In such a suspicious case, a doctor must be informed or the emergency doctor must come.
You can do that yourself
The problem with the treatment of idiopathic thrombocytopenic purpura is that the same drugs have different effectiveness in terms of curing the disease in different patients. Therefore, a high degree of cooperation is required from patients when it comes to choosing effective drugs. Self-healing processes also play a major role in the disease.
First of all, it is important for the chances of recovery that the patients see a doctor as quickly as possible. However, many sufferers notice the disease relatively late. Medical treatment often includes hospital stays. The patients follow the instructions of the nursing assistants and the doctors.
It makes sense if the affected patients support the drug therapy with a healthy lifestyle for which they are responsible. In addition, those affected always pay attention to potential complications and carefully observe their body feeling. In some cases, idiopathic thrombocytopenic purpura causes bleeding in the stomach or brain. Both complications pose a threat to the life of the person. Therefore, even in suspected cases of this kind, a doctor must be consulted immediately or an ambulance called.
