Essential thrombocytopenia

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 & 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.

Laboratory testing

The doctor will order a complete blood count (CBC), a test of clotting time, a bone marrow test, and a test for antiplatelet antibodies if it is available in the hospital laboratory. Patients with ITP usually have platelet counts below 20,000/mm3 and prolonged bleeding time. The size and appearance of the platelets may be abnormal. The red blood cell count (RBC) and white blood cell count (WBC) are usually normal, although about 10% of patients with ITP are also anemic. The blood marrow test yields normal results. Detection of antiplatelet antibodies in the blood is considered to confirm the diagnosis of ITP.

Treatment

General care and monitoring

There is no specific treatment for ITP. In most cases, the disorder will resolve without medications or surgery within two to six weeks. Nosebleeds can be treated with ice packs when necessary.

General care includes explaining ITP to the patient and advising him or her to watch for bruising, petechiae, or other signs of recurrence. Children should be discouraged from rough contact sports or other activities that increase the risk of trauma. Patients are also advised to avoid using aspirin or ibuprofen (Advil, Motrin) as pain relievers because these drugs lengthen the clotting time of blood.

Emergency treatment

Patients with acute ITP who are losing large amounts of blood or bleeding into their central nervous system require emergency treatment. This includes transfusions of platelets, intravenous immunoglobulins, or prednisone. Prednisone is a steroid medication that decreases the effects of antibody on platelets and eventually lowers antibody production. If the patient has a history of ITP that has not responded to prednisone or immunoglobulins, the surgeon may remove the patient's spleen. This operation is called a splenectomy. The reason for removing the spleen when ITP does not respond to other forms of treatment is that the spleen sometimes keeps platelets out of the general blood circulation.

Medications and transfusions

Patients with chronic ITP can be treated with prednisone, immune globulin, or large doses of intravenous gamma globulin. Although 90% of patients respond to immunoglobulin treatment, it is very expensive. About 80% of patients respond to prednisone therapy. Platelet transfusions are not recommended for routine treatment of ITP. If the patient's platelet level does not improve within one to four months, or requires high doses of prednisone, the doctor may recommend splenectomy. All medications for ITP are given either orally or intravenously; intramuscular injection is avoided because of the possibility of causing bleeding into the skin.

Surgery

Between 80% and 85% of adults with ITP have a remission of the disorder after the spleen is removed. Splenectomy is usually avoided in children younger than five years because of the increased risk of a severe infection after the operation. In older children, however, splenectomy is recommended if the child has been treated for 12 months without improvement; if the ITP is very severe or the patient is getting worse; if the patient begins to bleed into the head or brain; and if the patient is an adolescent female with extremely heavy periods.

Prognosis

The prognosis for recovery from acute ITP is good; 80% of patients recover without special treatment. The prognosis for chronic ITP is also good; most patients experience long-term remissions. In rare instances, however, ITP can cause life-threatening hemorrhage or bleeding into the central nervous system.

Key Terms

Autoimmune disorder

A disorder in which the patient's immune system produces antibodies that destroy some of the body's own products. ITP in adults is thought to be an autoimmune disorder.

Idiopathic

Of unknown cause. Idiopathic refers to a disease that is not preceded or caused by any known dysfunction or disorder in the body.

Petechiae

Small pinpoint hemorrhages in skin or mucous membranes caused by the rupture of capillaries.

Platelet

A blood component that helps to prevent blood from leaking from broken blood vessels. ITP is a bleeding disorder caused by an abnormally low level of platelets in the blood.

Prednisone

A corticosteroid medication that is used to treat ITP. Prednisone works by decreasing the effects of antibody on blood platelets. Long-term treatment with prednisone is thought to decrease antibody production.

Purpura

A skin discoloration of purplish or brownish red spots caused by bleeding from broken capillaries.

Splenectomy

Surgical removal of the spleen.

Thrombocytopenia

An abnormal decline in the number of platelets in the blood.

Further Reading

For Your Information

Books

• "Blood Component Therapy: Platelet Transfusions." In Internal Medicine On Call, edited by Steven A. Haist, et al. Stamford, CT: Appleton & Lange, 1991.
• Hays, Taru. "Hematologic Disorders." In Handbook of Pediatrics, edited by Gerald B. Merenstein, et al. Norwalk, CT: Appleton & Lange, 1994.
• "Hematology and Oncology: Immunologic Idiopathic Thrombocytopenic Purpura (ITP)." In The Merck Manual of Diagnosis and Therapy,, edited by Robert Berkow, et al. Rahway, NJ: Merck Research Laboratories, 1992.
• "Idiopathic Thrombocytopenic Purpura." In Professional Guide to Diseases, edited by Stanley Loeb, et al. Springhouse, PA: Springhouse Corporation, 1991.
• Linker, Charles A. "Blood." In Current Medical Diagnosis & Treatment 1998, edited by Lawrence M. Tierney, Jr., et al. Stamford, CT: Appleton & Lange, 1998.
• McMillan, Robert. "Platelet-Mediated Bleeding Disorders." In Conn's Current Therapy, edited by Robert E. Rakel, MD. Philadelphia: W. B. Saunders Company, 1998.
• "On-Call Problems: Thrombocytopenia." In Internal Medicine On Call, edited by Steven A. Haist, et al. Stamford, CT: Appleton & Lange, 1991.

Gale Encyclopedia of Medicine. Gale Research, 1999.