Idiopathic Thrombocytopenic Purpura Health Article

Definition

Idiopathic thrombocytopenic purpura, or ITP, is a bleeding disorder caused by an abnormally low level of platelets in the patient's blood. Platelets are small plate-shaped bodies in the blood that combine to form a plug when a blood vessel is injured. The platelet plug then binds certain proteins in the blood to form a clot that stops bleeding. ITP's name describes its cause and two symptoms. Idiopathic means that the disorder has no apparent cause. ITP is now often called immune thrombocytopenic purpura rather than idiopathic because of recent findings that ITP patients have autoimmune antibodies in their blood. Thrombocytopenia is another word for a decreased number of blood platelets. Purpura refers to a purplish or reddish-brown skin rash caused by the leakage of blood from broken capillaries into the skin. Other names for ITP include purpura hemorrhagica and essential thrombocytopenia.

Description

ITP may be either acute or chronic. The acute form is most common in children between the ages of two and six years; the chronic form is most common in adult females between 20 and 40. Between 10% and 20% of children with ITP have the chronic form. ITP does not appear to be related to race, lifestyle, climate, or environmental factors.

ITP is a disorder that affects the overall number of blood platelets rather than their function. The normal platelet level in adults is between 150,000 and 450,000/mm3. Platelet counts below 50,000 mm3 increase the risk of dangerous bleeding from trauma; counts below 20,000/mm3 increase the risk of spontaneous bleeding.

Causes and Symptoms

In adults, ITP is considered an autoimmune disorder, which means that the body produces antibodies that damage some of its own products—in this case, blood platelets. Some adults with chronic ITP also have other immune system disorders, such as systemic lupus erythematosus (SLE). In children, ITP is usually triggered by a virus infection, most often rubella, chickenpox, measles, cytomegalovirus, or Epstein-Barr virus. It usually begins about two or three weeks after the infection.

Acute ITP

Acute ITP is characterized by bleeding into the skin or from the nose, mouth, digestive tract, or urinary tract. The onset is usually sudden. Bleeding into the skin takes the form of purpura or petechiae. Purpura is a purplish or reddish-brown rash or discoloration of the skin; petechiae are small round pinpoint hemorrhages. Both are caused by the leakage of blood from tiny capillaries under the skin surface. In addition to purpura and petechiae, the patient may notice that he or she bruises more easily than usual. In extreme cases, patients with ITP may bleed into the lungs, brain, or other vital organs.

Chronic ITP

Chronic ITP has a gradual onset and may have minimal or no external symptoms. The low platelet count may be discovered in the course of a routine blood test. Most patients with chronic ITP, however, will consult their primary care doctor because of the purpuric skin rash, nosebleeds, or bleeding from the digestive or urinary tract. Women sometimes go to their gynecologist for unusually heavy or lengthy menstrual periods.

The risk factors for the development of chronic ITP include:

• female sex
• age over 10 years at onset of symptoms
• slow onset of bruising
• presence of other autoantibodies in the blood

Diagnosis

ITP is usually considered a diagnosis of exclusion, which means that the doctor arrives at the diagnosis by a process of ruling out other possible causes. If the patient belongs to one or more of the risk groups for chronic ITP, the doctor may order a blood test for autoantibodies in the blood early in the diagnostic process.

Physical examination

If the doctor suspects ITP, he or she will examine the patient's skin for bruises, purpuric areas, or petechiae. If the patient has had nosebleeds or bleeding from the mouth or other parts of the body, the doctor will examine these areas for other possible causes of bleeding. Patients with ITP usually look and feel healthy except for the bleeding.

The most important features that the doctor will be looking for during the physical examination are the condition of the patient's spleen and the presence of fever. Patients with ITP do not have fever whereas patients with lupus and some other types of thrombocytopenia are usually feverish. The doctor will have the patient lie flat on the examining table in order to feel the size of the spleen. If the spleen is noticeably enlarged, ITP is not absolutely ruled out but is a less likely diagnosis.