Published on Jan 14, 2020 and last reviewed on Jan 17, 2020 - 4 min read
Abstract
A blood disorder with abnormally low platelets resulting in bruising excessive bleeding is idiopathic thrombocytopenic purpura. Here, in this article, we discussed the types, symptoms, causes, risk factors, complications and treatment of ITP in detail.
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 level 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. Now 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 are numerous purple bruises the skin and mucous membranes. Sometimes, these bruises look like petechiae (pinpoint red or purple dots) on the skin, which look like rashes. Younger women and older men are more susceptible to this condition. Children become more susceptible to get ITP after a viral infection, such as chickenpox, measles, and mumps.
Treatment is usually not needed if the patient does not have signs of bruising and if the platelet count is not too low. Medications to increase the platelet count or spleen removal surgery might be necessary for more severe cases.
The types of ITP are:
Acute (short term) ITP - it lasts for six months or less and is the most common blood disorder in kids.
Chronic (long term) ITP - it lasts longer than six months and commonly affects adults.
Primary ITP - it occurs on its own.
Secondary ITP - it occurs with another condition.
Some patients do not have symptoms, but if they do, the common signs and symptoms of idiopathic thrombocytopenic purpura are:
Easy bruising.
Petechiae (pinpoint bruising), commonly on the legs.
Severe bleeding during surgery.
Spontaneous and frequent nosebleeds.
Blood in the stool.
Heavy menstruation.
Prolonged bleeding from small cuts.
Gum bleeding.
Blood in the urine.
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 now. It has been found that the immune system destroys platelets by producing antibodies. Then these platelets are removed by the spleen, which results in low platelet count. It is also believed that the immune system affects platelet production. ITP usually develops following a viral infection in kids.
Risk Factors:
Autoimmune diseases (rheumatoid arthritis, lupus).
Chronic infections.
Some types of cancer.
Idiopathic Thrombocytopenic Purpura and 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.
If you are experiencing symptoms of ITP, your doctor will take a complete medical history and perform physical examinations. Then if needed, the doctor will suggest you to get the following tests done:
Complete blood count - to check for low platelet count.
Liver and kidney function test.
A blood test to check for platelet antibodies.
Bone marrow test - in case your 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.
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 also, treatment is no needed. But, the doctor will monitor the platelet and other blood cell count to check if you would have to be put under treatment. The treatment options in symptomatic patients are:
Medications:
Corticosteroids - Prednisone is used to increase your platelet count by inhibiting the activity of the immune system.
Intravenous immunoglobulin (IVIg) - In case of severe bleeding or if the patient needs surgery, then intravenous immunoglobulin is given to increase platelet count in a short time.
Anti-D Immunoglobulin - For patients with Rh-positive blood, anti-D immunoglobulin is used to increase platelet count.
Rituximab - This medicine destroys the immune cells that destroy platelets.
Thrombopoietin receptor agonists (Romiplostim, Eltrombopag) - Promotes bone marrow to produce more platelets, which prevents bruising and spontaneous bleeding.
Other immunosuppressants (Cyclophosphamide, Azathioprine, Mycophenolate) - They inhibit the activity of the immune system.
Antibiotics - To treat Helicobacter pylori infection, which is a bacterial infection associated with ITP.
In patients who have severe ITP and if medicines are not helping, 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 all of a sudden. Getting your spleen removed increases the risk of infections.
You might be advised to make some lifestyle modifications like:
Do not take drugs that affect blood clotting, such as Aspirin, Ibuprofen, and blood thinners like Warfarin and Heparin.
Avoid alcohol consumption.
Avoid sports and high-impact activities that can result in injury.
Choose low-impact activities.
The possible complications include:
Severe bleeding.
Bleeding into the brain (can be fatal).
But the prolonged use of corticosteroids can result in:
Osteoporosis.
Infections.
Cataracts.
Loss of muscle mass.
For more information on ITP, consult a hematologist now.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
The drugs associated with Thrombotic thrombocytopenic purpura (TTP) are Quinine, Ticlopidine, Mitomycin, Cyclosporin, Clopidogrel, Platelet aggregation inhibitors, Antibiotics, Antineoplastic, Immunosuppressive agents, and Muscle relaxants.
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).
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.
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.
Last reviewed at:
17 Jan 2020 - 4 min read
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Dr. Goswami Parth Rajendragiri
Pathology
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