Idiopathic Thrombocytopenic Purpura (ITP) - Types, Symptoms, Causes, Diagnosis, and Treatment
Published on Jan 14, 2020 and last reviewed on Dec 15, 2022 - 5 min read
Abstract
A blood disorder with abnormally low platelets resulting in bruising and excessive bleeding is idiopathic thrombocytopenic purpura. Read to know more.
What Is Idiopathic Thrombocytopenic Purpura (ITP)?
Idiopathic thrombocytopenic purpura, now called ITP or immune thrombocytopenia, is an immune blood disorder where the blood does not clot properly. It can result in severe and spontaneous bruising and bleeding due to low levels of platelets in the blood.
The bone marrow is responsible for platelet production, and these platelets clump together to stop bleeding by forming a blood clot. The blood clot seals the small tear in the blood vessel walls. If the platelet level is low, it results in slow blood clot formation and prolonged bleeding. This condition also increases the risk of internal bleeding or bleeding under the skin.
ITP patients have purpura, which is numerous purple bruises on the skin and mucous membranes. Sometimes, these bruises look like petechiae (pinpoint red or purple dots) on the skin, which resembles rashes. Younger women and older men are more susceptible to this condition. Children become more susceptible to ITP after viral infections like chickenpox, measles, and mumps.
Treatment is usually not needed if the patient does not have signs of bruising and the platelet count is not too low. Medications to increase the platelet count or spleen removal surgery might be necessary for more severe cases.
What Are the Types of Idiopathic Thrombocytopenic Purpura?
The types of ITP are:
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Acute (Short-Term) ITP - It lasts six months or less and is the most common blood disorder in children of ages two to six years. It generally starts suddenly, and the symptoms disappear in less than six months or within weeks.
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Chronic (Long-Term) ITP - It lasts longer than six months and commonly affects adults. The onset of this type can happen at any age, and it can last several years or even a lifetime. Adults are more prone to this type than children. The recurrence rate is high for chronic ITP.
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Primary ITP - Primary ITP occurs in isolation. It occurs in the absence of other medical conditions associated with thrombocytopenia.
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Secondary ITP - It occurs with another underlying condition, such as hepatitis C infection or systemic lupus erythematosus (a condition in which the immune system attacks its own cells).
What Are the Symptoms of Idiopathic Thrombocytopenic Purpura?
Some patients do not have symptoms, but if they do, the common signs and symptoms of idiopathic thrombocytopenic purpura include:
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Easy bruising.
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Petechiae (pinpoint bruising), commonly on the legs.
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Severe bleeding during surgery.
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Spontaneous and frequent nosebleeds.
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Blood in the stool.
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Heavy menstruation.
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Prolonged bleeding from small cuts.
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Gum bleeding.
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Blood in the urine.
What Causes Idiopathic Thrombocytopenic Purpura?
Idiopathic means of unknown cause. Because the cause was unclear in the past, it was named idiopathic thrombocytopenic purpura. But now it is clear that the immune system results in this condition, which is why it is called immune thrombocytopenia. It has been found that the immune system destroys platelets by producing antibodies. Then these platelets are removed by the spleen, which results in a low platelet count. It is also believed that the immune system affects platelet production. ITP usually develops following a viral infection in kids.
What Are the Risk Factors for Idiopathic Thrombocytopenic Purpura?
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Autoimmune diseases such as rheumatoid arthritis (an autoimmune disease of joints) or systemic lupus erythematosus.
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Chronic infections.
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Some types of cancer.
How to Manage Idiopathic Thrombocytopenic Purpura in Pregnancy?
In pregnant women, if the platelet count is not very low, then treatment is not required, and only regular monitoring will do. But if the counts are very low, it can result in severe bleeding during and after delivery. Consult a doctor to come up with a treatment plan.
How Is Idiopathic Thrombocytopenic Purpura Diagnosed?
If a person is experiencing symptoms of ITP, the doctor will take a complete medical history and perform physical examinations. Then if needed, the doctor will suggest getting the following tests done:
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Complete Blood Count (CBC) - This is to check for low platelet count.
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Liver and kidney function test.
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A blood test to check for platelet antibodies.
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Bone marrow test - In case the platelet count is low, a bone marrow test might be needed.
In ITP patients, the bone marrow will be normal, as platelets are destroyed in the blood and spleen after they leave the bone marrow.
What Are the Treatment Options for Idiopathic Thrombocytopenic Purpura?
Treatment is not usually necessary for kids with acute ITP, as it will heal without treatment in about six months. In adults with no symptoms or almost normal platelet count, treatment is unnecessary. But, the doctor will monitor the platelet and other blood cell count to check if treatment is needed. The treatment options in symptomatic patients are:
Medications:
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Corticosteroids - Prednisone is used to increase the platelet count by inhibiting the activity of the immune system.
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Intravenous Immunoglobulin (IV Ig) - In case of severe bleeding or if the patient needs surgery, then intravenous immunoglobulin is given to increase platelet count in a short time.
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Anti-D Immunoglobulin - For patients with Rh-positive blood, anti-D immunoglobulin is used to increase platelet count.
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Rituximab - This medicine destroys the immune cells that attack platelets.
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Thrombopoietin Receptor Agonists (Romiplostim, Eltrombopag) - Promotes bone marrow to produce more platelets, which prevents bruising and spontaneous bleeding.
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Immunosuppressants (Cyclophosphamide, Azathioprine, Mycophenolate) - They inhibit the activity of the immune system.
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Antibiotics - To treat Helicobacter pylori infection, which is a bacterial infection associated with ITP.
Surgery:
In patients who have severe ITP and if medicines are not helpful, then the doctor will suggest surgical removal of the spleen (splenectomy). This surgery is not performed in kids because most children get better on their own. Getting the spleen removed increases the risk of infections.
Lifestyle Alterations:
Patients might be advised to make some lifestyle modifications like:
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Avoid taking drugs that can affect coagulation (blood clotting), such as Aspirin, Ibuprofen, and blood thinners like Warfarin and Heparin.
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Avoid alcohol consumption.
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Avoid sports and high-impact activities that can result in injury.
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Choose low-impact activities.
What Are the Complications of Idiopathic Thrombocytopenic Purpura?
The possible complications include:
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Severe bleeding.
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Bleeding into the brain (can be fatal).
But the prolonged use of corticosteroids can result in:
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Osteoporosis (a condition characterized by brittle and weak bones).
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Infections.
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Cataracts.
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Loss of muscle mass.
Conclusion:
Idiopathic thrombocytopenic purpura is characterized by easy bleeding due to unusually low platelet count. Children who develop ITP often recover on their own, but in adults, this condition is usually long-term. Patients with symptoms require treatment which may include medications or surgery. Certain precautions and preventive measures, if taken properly, can help prevent this condition.
Frequently Asked Questions
1.
Can ITP Lead to More Serious Blood Disorders?
ITP is the immunologic destruction of platelets, and it does not turn into serious blood disorders like aplastic anemia or leukemia. Although, aplastic anemia is characterized by pancytopenia, which includes anemia, thrombocytopenia, and leukopenia.
2.
How Can We Cure ITP?
Spontaneous recovery occurs in 90% of cases of acute ITP, while only less than 10% of cases of chronic ITP recover spontaneously. The life expectancy depends on the treatment plan and reduced risk for life-threatening complications.
3.
What Are the Treatment Modalities for ITP?
Treatment is directed at reducing the level and source of autoantibodies and reducing the rate of destruction of sensitized platelets. This is possible by corticosteroid therapy, immunosuppressive drugs, and splenectomy. The beneficial effects of splenectomy in chronic ITP are due to the removal of the major source of autoantibody synthesis. Platelet transfusions are helpful as a palliative measure only in patients with severe hemorrhage.
4.
Does ITP Compromise the Immune System?
Idiopathic Thrombocytopenic Purpura is also known as immune thrombocytopenic purpura, and it is characterized by immunologic destruction of platelets and normal or increased megakaryocytes in the bone marrow. It is an autoimmune disorder in which antibodies (IgG) are produced against platelet antigens. The antibody-coated platelets are destroyed by phagocytic cells in the spleen.
5.
Can Stress Affect the Platelet Count?
Stress usually causes an increase in platelet count. Stressful life events and anxiety can lead to an imbalance in the immune system that contributes to the development of autoimmune diseases or suppressed immune responses. Physical, physiological, and resultant oxidative stress in the body triggers the episodes of ITP. ITP patients appear to face greater levels of fear and are more emotionally attacked than those without the disease.
6.
Does ITP Lowers Life Expectancy?
Life expectancy depends on the treatment plan. Chronic ITP occurs more commonly in adults, particularly in women of child-bearing age (20 to 40 years). The disorder develops insidiously and persists for several years. Though chronic ITP is idiopathic, similar immunologic thrombocytopenia may be seen in association with AIDS and autoimmune thyroiditis.
7.
What Foods Should Be Avoided if You Have a Low Platelet Count?
Certain foods have blood-thinning effects, so we should avoid certain foods if we have ITP. They are meat, fast food, canned food, garlic, onion, saturated fats found in whole dairy products, non-plant-based oils, such as butter and margarine, tomatoes, berries, food in boxed and frozen food aisles.
8.
Does ITP Go Away?
ITP is the immunologic destruction of platelets. For most adults with ITP, the condition is chronic (lifelong). ITP may happen suddenly and go away in about six months, or it may be ongoing if chronic and last for years. Treating ITP helps to come out of the disorder.
9.
Does ITP Get Worse Over Time?
Chronic ITP is explained by the formation of anti-platelet autoantibodies, usually by platelet-associated IgG humoral antibodies synthesized mainly in the spleen. Some of the antibodies directed against platelet surfaces also interfere in their function. Sensitized platelets are destroyed mainly in the spleen and rendered susceptible to phagocytosis by cells of the reticuloendothelial system. But unlike autoimmune disorders, it is not progressive, but the risk of bleeding increases.
10.
Can You Die From Low Platelet Count?
In a healthy body, the lifespan of platelets is about ten days. When there is internal bleeding, the platelet count falls below 10,000 per microliter. Severe thrombocytopenia can cause bleeding in the brain, which can be fatal.
11.
What Are the Causes of Thrombocytopenia?
The most common causes of thrombocytopenia are aplastic anemia, leukemia, myelofibrosis, megaloblastic anemia, marrow infiltrations. The drugs such as Quinine, Quinidine, Sulfonamides, Rifampicin, Anticancer drugs, Diclofenac, Acyclovir. Massive transfusion of old stored blood to bleeding patients, splenomegaly, alcohol intake, accelerated platelet destruction.
12.
What Are the Drugs Associated With TTP?
The drugs associated with Thrombotic thrombocytopenic purpura (TTP) are Quinine, Ticlopidine, Mitomycin, Cyclosporin, Clopidogrel, Platelet aggregation inhibitors, Antibiotics, Antineoplastic, Immunosuppressive agents, and Muscle relaxants.
13.
What Is the Effect of Honey on Human Platelets?
Honey showed moderate inhibition of platelet aggregation. The coagulation assay showed that at higher concentrations, honey samples increased whole blood clotting time. When in platelet-poor plasma (PPP), honey samples prolonged prothrombin time (PT), thrombin time (TT).
14.
Can Low Platelets be Genetic?
In inherited thrombocytopenia or familial thrombocytopenia, the low platelets are formed by a genetic mutation and not by autoantibodies, as in the case with primary ITP. But currently, it is not usually considered an inherited disease unless multiple family members are diagnosed with ITP.
15.
What Is Acute ITP?
Acute ITP is a self-limited disorder seen most frequently in children following recovery from a viral illness such as hepatitis C, infectious mononucleosis, HIV infection, or an upper respiratory illness. The onset of acute ITP is sudden, but recovery occurs within a few weeks to 6 months.
- Idiopathic thrombocytopenic purpura
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4147825/ - Immune thrombocytopenic purpura (ITP)
https://medlineplus.gov/ency/article/000535.htm - Idiopathic thrombocytopenic purpura (ITP) - new era for an old disease
https://pubmed.ncbi.nlm.nih.gov/31199777/
Last reviewed at:
15 Dec 2022 - 5 min read
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Dr. Goswami Parth Rajendragiri
Pathology
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