Immune thrombocytopenic purpura
Immune thrombocytopenic purpura, also known as idiopathic thrombocytopenic purpura and referred to as ITP, is a disease affecting both children and adults which involves the circulatory system, specifically blood platelets. Characterized by easy bruising and bleeding, the disease can be fatal in rare cases.
Definition & Facts
Immune thrombocytopenic purpura is a disorder of the blood which is characterized by a substantially reduced number of circulating platelets (thrombocytopenia). This disorder presents as a single diagnosis in otherwise healthy adults and children and causes severe and excessive bruising.
Bleeding occurs when the platelet levels are very low. However, most children make a full recovery. Adults with the disorder are not likely to recover and live with the disorder long term. Depending on the severity of the disorder, the patient may or may not need treatment. If bleeding is present, treatment is needed, especially in rare cases in which internal bleeding occurs.
Symptoms & Complaints
Patients having ECG (electrocardiograph) performed with suction connections may form petechiae. If the patient is bedridden, they will form on the back. The severity of symptoms presenting is an indicator of the severity of disorder.
The disorder is caused by the immune system malfunctioning and recognizing platelets as an invader. This recognition causes the immune system to attack blood platelets and destroy them. Sometimes the cause of the immune response can be determined, but if not the disorder is referred to as idiopathic.
Immune thrombocytopenic purpura can develop in children after fighting long-term viral infections such as mumps or measles. It is believed in these cases the infection has triggered the immune response. In adults viral infections such as hepatitis C infection and HIV may cause the disorder.
Patients that have had septicemia (a bacterial infection of the blood) may also develop the disorder. Additionally patients diagnosed with a bacterial infection, Heliobacter pylori infection commonly referred to as H. pylori, may develop ITP.
Additionally, ITP can develop in patients taking certain heart medications and blood thinners, seizure medications, and chemotherapy are at risk for developing the disorder. ITP may have a genetic factor and has been known to be present in multiple generations of a family.
Diagnosis & Tests
Immune thrombocytopenic purpura is diagnosed first by a physical examination of the bruising and bleeding. A large part of the diagnosis involves ruling out other diseases and disorders.
Blood is drawn and it is run through several diagnostic tests including a CBC, or complete blood count, to determine the numbers and types of cells present. When examined under a microscope, a positive smear will show normal neutrophils and red blood cells but will have a marked decrease in platelets and white blood cells. Positive smears will not have clumping of platelets found in normal blood smears.
When diagnosing accompanying bleeding in the brain (cerebral hemorrhage) caused by the disease, the health care provider may assess the presence of hypertension and headache. When looking in the eye, the edges of the optic disc may be blurred and there may be bleeding of the retina.
Some doctors may order a bone marrow test to try to identify what is the cause of the low platelet count. This test is not recommended for children. Some physicians test the bone marrow, others do a liquid test and only collect fluid in the marrow or the doctor may order both. Because platelets are destroyed in the blood, there should be a normal amount in the marrow if the disorder is immune thrombocytopenic purpura.
Treatment & Therapy
Many patients diagnosed with immune thrombocytopenic purpura will not need treatment. Children are remarkably resilient and their bodies are able to return to normal over time if the disorder is not severe. Regular monitoring of blood platelets helps determine if treatment is needed.
In patients where ITP has become chronic, treatments are available, but many patients find the side effects of the medications more intolerable than the disease and forego treatment. For those that do respond to treatment, medications such as corticosteroids are the most prescribed drug used to treat ITP.
Other treatments include injections of immunoglobulin therapy (IVIG) which may work by inhibiting the receptors on phagocytes that attack platelets. Drugs that help increase platelet production, thrombopoietin receptor agonists, are also used. Drugs that suppress the immune system may also be used.
Cases that are resistant to treatment may be treated surgically by removing the spleen (splenectomy). This procedure is reserved for patients that are resistant to treatment. Removing the spleen removes the source of platelet destruction but leaves the patient with a permanently weakened immune system, putting them at risk of infection.
Prevention & Prophylaxis
Patients need to avoid bruising so lifestyle changes may need to occur. For those involved in sports like karate, boxing and football where rough physical contact occurs, these activities should be discontinued.
Preventing traumatic brain injury via hits to the head is even more important, so taking care to prevent falling may be a life saving behavior as injury to the head could lead to bleeding on the brain.
Additionally, patients need to monitor their health and contact their physician at the first signs of an infection. Fever is an indication that infection is present and must be reported. Living a healthy lifestyle and taking care to prevent lacerations particularly if one works in the kitchen with knives are important preventative measures.