About purpura, idiopathic thrombocytopenic

What is purpura, idiopathic thrombocytopenic?

Idiopathic thrombocytopenic purpura (ITP) is a not infrequent autoimmune bleeding disorder characterized by the abnormally low levels of blood cells called platelets, creating a condition known as thrombocytopenia. Platelets are specialized blood cells that help prevent and stop bleeding by inducing clotting. In many ITP cases, there are no readily apparent causes or underlying disease (idiopathic), but frequently there are associated collagen vascular diseases or underlying neoplasms, most frequently lymphoid. The cells of the immune system, lymphocytes, produce anti-platelet antibodies that attach to the platelets. The presence of antibodies on platelets leads to their destruction in the spleen. The disorder is characterized by abnormal bleeding into the skin resulting in bruising, which is what the term purpura means. Bleeding from mucous membranes also occurs, and may subsequently result in low levels of circulating red blood cells (anemia).

ITP presents as a brief, self-limiting form of the disorder (acute ITP) or a longer-term form (chronic ITP). Acute ITP accounts for about 50% of cases, and chronic ITP accounts for the remainder. Eighty percent (80%) of the children with ITP have the acute form while the chronic form affects mostly adults. The acute form usually resolves without treatment (spontaneously) within three to six months. When thrombocytopenia lasts for more than six to 12 months, ITP is classified as the chronic form. Onset of acute ITP is often rapid, while the onset of the chronic form may be gradual.

What are the symptoms for purpura, idiopathic thrombocytopenic?

Petechiae may look like a rash and usually appear in clusters. Here they appear on a leg (A) and on an abdomen (B).

Immune thrombocytopenia may have no signs and symptoms. When they do occur, they may include:

  • Easy or excessive bruising
  • Superficial bleeding into the skin that appears as pinpoint-sized reddish-purple spots (petechiae) that look like a rash, usually on the lower legs
  • Bleeding from the gums or nose
  • Blood in urine or stools
  • Unusually heavy menstrual flow

What are the causes for purpura, idiopathic thrombocytopenic?

Immune thrombocytopenia usually happens when your immune system mistakenly attacks and destroys platelets, which are cell fragments that help blood clot. In adults, this may be triggered by infection with HIV, hepatitis or H. pylori — the type of bacteria that causes stomach ulcers. In most children with ITP, the disorder follows a viral illness, such as the mumps or the flu.

What are the treatments for purpura, idiopathic thrombocytopenic?

People with mild immune thrombocytopenia may need nothing more than regular monitoring and platelet checks. Children usually improve without treatment. Most adults with ITP will eventually need treatment, as the condition often becomes severe or long term (chronic).

Treatment may include a number of approaches, such as medications to boost your platelet count or surgery to remove your spleen (splenectomy). Talk with your doctor about the risks and benefits of your treatment options. Some people find that the side effects of treatment are more burdensome than the effects of the disease itself.

Surgery

If your condition is severe or persists despite initial drug treatment, your doctor may suggest surgery to remove your spleen. This quickly eliminates the main source of platelet destruction in your body and improves your platelet count, though it doesn't work for everyone. Living without a spleen permanently increases your susceptibility to infection.

Emergency treatment

Although rare, severe bleeding can occur with ITP. Emergency care usually includes transfusions of platelet concentrates. Steroids and immune globulin may also be given through a tube in a vein.

What are the risk factors for purpura, idiopathic thrombocytopenic?

ITP is more common among young women. The risk appears to be higher in people who also have diseases such as rheumatoid arthritis, lupus and antiphospholipid syndrome.

Is there a cure/medications for purpura, idiopathic thrombocytopenic?

Medications

Your doctor will talk with you about over-the-counter medications or supplements you take and whether you need to stop using any that might inhibit platelet function. Examples include aspirin, ibuprofen (Advil, Motrin IB, others) and ginkgo biloba.

Medications to treat ITP may include:

  • Steroids. Your doctor will likely start you on an oral corticosteroid, such as prednisone. Once your platelet count is back to a safe level, you can gradually discontinue taking the drug under the direction of your doctor. Long-term use of these medications isn't recommended because they can increase your risk of infections, high blood sugar and osteoporosis.
  • Immune globulin. If corticosteroids don't help, your doctor may give you an injection of immune globulin. This drug may also be used if you have critical bleeding or need to quickly increase your blood count before surgery. The effect usually wears off in a couple of weeks.
  • Drugs that boost platelet production. Medications such as romiplostim (Nplate) and eltrombopag (Promacta) help your bone marrow produce more platelets. These types of drugs can increase your risk of blood clots.
  • Other drugs. Rituximab (Rituxan, Truxima) helps increase your platelet count by reducing the immune system response that's damaging your platelets. But this drug also can reduce the effectiveness of vaccinations, which may be needed if you later choose surgery to remove your spleen.

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