Idiopathic thrombocytopenic purpura is an autoimmunologic deficiency of platelets. Patients suffer from spontaneous bleeding. The condition is treated with medication, which leads to cure in 70 percent of all cases.
What is idiopathic thrombocytopenic purpura?
Thrombocytopenia is a deficiency of blood platelets. About 150,000 to 450,000 platelets are normally found in one centiliter of blood. A shortfall below these standard values is thrombocytopenia. The deficiency symptoms are caused either by reduced formation, disturbed platelet distribution or unnaturally high platelet breakdown. Bleeding tendencies are expected with plateletopenias below 80,000 per centiliter. Levels below 50,000 per centiliter cause spontaneous nosebleeds and hematomas, cerebral hemorrhage, and gastrointestinal bleeding. Idiopathic thrombocytopenic purpura (ITP) is also known as immune thrombocytopenia, purpura haemorrhagica, autoimmune thrombocytopenia, thrombocytopenic purpura, or immune thrombocytopenic purpura. This is an autoimmune disease that attacks the platelets. 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 disease, Werlhof-Wichmann syndrome or Werlhof disease. This variant preferentially affects women and occurs in about 100 new cases per year among one million inhabitants. Disease is also called Evans syndrome against a background of autoimmune hemolytic anemia.
Causes
Idiopathic thrombocytopenic purpura results from a misdirected immune process. Accordingly, the cause of the platelet deficiency is an autoimmune disorder. The patient’s immune system produces free and platelet-bound antibodies against adhesion molecules such as Gp IIb/IIIa, thereby shortening the life span of the platelets. As for all other autoimmune diseases, the primary cause of idiopathic thrombocytopenic purpura has not yet been conclusively determined. Different speculations are available as explanations. Meanwhile, it has been observed that the disease is often preceded by infections of the respiratory tract or those of the gastrointestinal tract. Often, infections with HIV or EBV are also present in the past. Therefore, it is now suspected that cross-reactive antigens of the viral pathogens cause antibody formation in the context of molecular mimicry, especially the formation of immunoglobulin G. Nevertheless, not all documented cases can be explained by infections. For a long time, it was not at all clear that the phenomenon was an autoimmune disease at all. Although the exact pathogenesis and cause of the disease have not been fully elucidated to date, scientists now agree at least on the autoimmunologic basis.
Symptoms, complaints, and signs
The complaints of patients with idiopathic thrombocytopenic purpura vary from case to case. Thus, the disease is associated with an extremely variable clinical picture and may cause quite a few symptoms in individual cases. The disease is always based on increased platelet degradation. On this basis, the platelets decrease. Since platelets play an important role in the coagulation cascade, the reduced platelet count results in a more or less severe bleeding tendency. Many patients spontaneously experience punctate bleeding of 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 has given the disease its name. In individual cases, spontaneous nosebleeds may also occur. Suffering women also often suffer from prolonged menstruation. Since the disease can also cause brain hemorrhages or bleeding processes in the gastrointestinal tract, life-threatening conditions result in extreme cases. In the case of cerebral hemorrhage, for example, intracranial pressure increases. The brain is compressed in the skull and can suffer permanent damage. Normally, clinically manifest symptoms do not occur until platelet levels are less than 30,000 per centiliter. Values of less than 10,000 platelets per centiliter cause life-threatening conditions.Nevertheless, life-threatening situations rarely occur in idiopathic thrombocytopenic purpura.
Diagnosis and course of the disease
Idiopathic thrombocytopenic purpura is determined by laboratory diagnosis against a background of medical history. In the history, past infections may provide a crucial clue. Blood testing demonstrates a deficiency of platelets. Similar symptoms to the disease are evoked by thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and heparin-induced thrombocytopenia. In the differential diagnosis, these diseases must be excluded. Ideally, the physician will encounter the presumed primary cause of infection during the diagnostic process. The prognosis is relatively good with cure rates of around 70 percent. However, about four percent die from cerebral hemorrhage.
Complications
In many cases, this disease is diagnosed late because there are no consistent or characteristic symptoms and complaints. Unfortunately, treatment cannot be given early for this reason, which limits the prospects for a complete cure. As a rule, however, there is a breakdown of platelets. As a result of this breakdown, affected individuals experience increased bleeding, which can occur in various 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, patients suffer from frequent nosebleeds. In the worst case, bleeding can occur in the brain or stomach, which can lead to death if left untreated. The bleeding in the brain causes the brain pressure to rise, resulting in 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 particular medication will lead to a cure in a patient or whether another must be taken as well. If left untreated, there is a reduction in life expectancy.
When should you see a doctor?
If bleeding occurs repeatedly for no apparent reason, a doctor is needed. If there is sudden blood in the stool or urine that occurs over several days or sporadically, there is reason for concern. A doctor’s visit is needed to determine the cause. Frequent unexpected bleeding from the nose or gums are indications of abnormalities that should be investigated. Bleeding of the skin, changes in skin appearance, and spontaneous onset of bleeding on the body are warning signs that need to be investigated. If bleeding can be difficult to stop, a physician should be informed of the observations. If cerebral hemorrhages occur, a life-threatening condition may develop. Therefore, a doctor’s visit is necessary as soon as dizziness, unsteadiness of gait, sleep disturbances or a general feeling of malaise occur. If disturbances of consciousness set in, a doctor must be consulted immediately. If there is a loss of consciousness, an emergency doctor must be called, as an acute condition has occurred that requires immediate action. First aid measures should be administered until the doctor arrives. Women should be examined if they suffer from heavy and at the same time prolonged menstrual bleeding. If menstruation lasts longer than 7 days, this is considered unusual and may be a signal of disturbances in the organism. In case of palpitations, changes in blood pressure or irregularities in the heart rhythm, a doctor must be consulted.
Treatment and therapy
In some cases of ITP, no therapy is needed because spontaneous healing occurs. If spontaneous healing does not occur, glucocorticoids are given. In addition, immunoglobulin therapy or intravenous administration of anti-D immunoglobulin is often given. In severe bleeding complications, splenectomy is performed because the immunologic dysfunction is usually expected to be in the spleen. In addition, transfusion of platelet foreign concentrates can be performed in bleeding complications. However, this procedure leads to worsening in some cases. Cytostatic drugs suppress autoantibody-producing cells. Alternatively, monoclonal antibodies may be administered in combination with immunoglobulins. In addition, administration of a nonstructural analog 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 regular controls. The treatment plan is usually changed several times in the therapy of ITP before decisive successes become apparent. Some scientists assume that the 70 percent of cured patients were cured from thrombocytonpenia exclusively by self-healing. In fact, against the background of self-healing, it is difficult to assess individual drugs for their true efficacy.
Prospect and prognosis
Idiopathic thrombocytopenic purpura – now more commonly referred to as immune thrombocytopenia (ITP) – usually has a good prognosis in adults. A prerequisite for cure, however, is that an optimal course of therapy can be achieved. In addition, cerebral hemorrhage must not occur. In adults, 70 to 80 percent of idiopathic thrombocytopenic purpura can be cured. However, it is also a fact that the outlook is worse if recurrences occur. In children, the course of idiopathic thrombocytopenic purpura is somewhat different. In younger children, idiopathic thrombocytopenic purpura is more likely to occur as an acute illness. Frequently, immune thrombocytopenia occurs after an infection. The chronic form of idiopathic thrombocytopenic purpura is more rare in young children and adolescents. Therefore, the course of the disease is different. Overall, the prognosis is better than in adults. Provided that only mild bleeding occurs, therapeutic intervention may not be necessary. In children, even spontaneous cures are possible. A certain number of children and adolescents die as a result of intracerebral hemorrhage. The overall mortality rate for Idiopathic Thrombocytopenic Purpura is 4 percent of those affected. Prognosis is poor if cerebral hemorrhage occurs during the course of the disease. In recurrences, ITP is sometimes improved by splenectomy. However, recurrences can also occur after surgery.
Prevention
Idiopathic thrombocytopenic purpura appears to be a late consequence of infections. Thus, the phenomenon could be prevented, at least to some extent, by general infection prophylaxis.
Follow-up
Follow-up of idiopathic thrombocytopenic purpura depends on the age of the patient and the individual course. If it is a late sequela after infection, individuals can take preventive measures to reduce the risk of recurrence. However, with the same therapy, patients may respond differently to medications, which affects the healing process. For this reason, sufferers should be in close contact with their physician and help select medications. The body’s self-healing powers also play a crucial role in the recovery process. These require medical support. It is therefore important for patients to make an appointment with their doctor at an early stage if any abnormalities occur. During their stay in the hospital and also in the subsequent phase, patients should observe themselves and follow medical orders. Complementarily, they can adjust their lifestyle to stabilize their health. They strengthen their fitness through a nutritious diet and sufficient activities. When it comes to patient self-responsibility, timely detection of possible complications is also an important issue. For example, sudden bleeding can be detected at an early stage. In such a suspected case, a doctor must be informed or the emergency physician must come.
What you can do yourself
In the treatment of Idiopathic Thrombocytopenic Purpura, the problem is that the same drugs have different efficacies in different patients in terms of curing the disease. Therefore, a high degree of cooperation is required from patients when it comes to selecting effective drugs. In addition, self-healing processes play a major role in the disease. It is important for the chances of recovery that patients see a doctor as soon as possible. However, many sufferers notice the disease relatively late. Medical treatment often includes hospitalization. In this case, the patients follow the orders of the nursing assistants as well as the doctors. It makes sense for the affected patients to support the drug therapy with a healthy lifestyle for which they themselves are responsible.In addition, sufferers always watch out for potential complications and keep a close eye on their bodies. This is because in some cases, Idiopathic Thrombocytopenic Purpura causes bleeding in the stomach or brain. Both of these complications pose a threat to the life of the individuals. Therefore, even in such suspected cases, a physician should be consulted immediately or an emergency physician should be called.