About nonthrombocytopenic idiopathic purpura

What is nonthrombocytopenic idiopathic purpura?

Henoch-Schonlein purpura is a rare inflammatory disease of the small blood vessels (capillaries) and is usually a self-limited disease. It is the most common form of childhood vascular inflammation (vasculitis) and results in inflammatory changes in the small blood vessels. The symptoms of Henoch-Schonlein purpura usually begin suddenly and may include headache, fever, loss of appetite, cramping abdominal pain, and joint pain. Red or purple spots typically appear on the skin (petechial purpura). Inflammatory changes associated with Henoch-Schonlein purpura can also develop in the joints, kidneys, digestive system, and, in rare cases, the brain and spinal cord (central nervous system).

In one form of the disorder, termed Schonlein's purpura, the skin and joints are affected but the gastrointestinal tract is not. In another form, known as Henoch's purpura, affected individuals have purplish spots on the skin and acute abdominal problems. People with Henoch's purpura are not affected by joint disease.

The exact cause of Henoh-Schonlein purpura is not fully understood, although research suggests that it may be an autoimmune disease or, in some rare cases, an extreme allergic reaction to certain offending substances (e.g., foods or drugs).

What are the symptoms for nonthrombocytopenic idiopathic purpura?

Diarrheablood in eye symptom was found in the nonthrombocytopenic idiopathic purpura condition

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 nonthrombocytopenic idiopathic purpura?

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 nonthrombocytopenic idiopathic purpura?

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.

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.

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 nonthrombocytopenic idiopathic purpura?

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 nonthrombocytopenic idiopathic purpura?

Nonthrombocytopenic idiopathic purpura is a generally self-limited and inflammatory disease of small blood vessels.

  • In mild conditions, no treatment requires just regular monitoring and platelet checking needed, and without treatment, it improves in children, but in some cases, it takes long-term treatment.
  • In that case, some medications are required and some surgeries are also required.
  • Some medications need to be stopped like aspirin, ibuprofen (Advil, Motrin IB, others) and ginkgo Biloba.


Following are some medications that treat this disease:

  • Steroid: an oral dosage of corticosteroid, such as prednisone, is advised by doctors at the initial level to boost your platelets count but after it reaches curtail levels, the dosage is reduced step by step, because longer uses of this will create contradictions like infections, high blood sugar, and osteoporosis.
  • Immune globulin: if steroid doesn’t work, then doctors will give the injection dosage of Immune globulin that will boost your blood count effectively.
  • Drugs that boost platelet production: romiplostim (Nplate) and eltrombopag (Promacta). These medicines help your born marrow produce more platelets.
  • Rituximab (Rituxan, Truxima) is also used for treatment, and sometimes surgery and emergency treatments also can be done by doctors.


Symptoms
Malaise,Headache,Fever,Rash,Red spotting of the skin (most often on the buttocks and backs of the legs),Cramping abdominal pain,Blood may be present in the stool and abnormal bleeding,Joint pain,Vomiting,Diarrhea
Conditions
Diffuse erythema,Arthralgia,Melena,Petechiae
Drugs
Glucocorticoids (steroid) drugs such as prednisone,Dapsone

Video related to nonthrombocytopenic idiopathic purpura