Idiopathic Thrombocytopenic Purpura (ITP)

Picture of Idiopathic Thrombocytopenic Purpura (ITP)

Idiopathic thrombocytopenic purpura (ITP), also known as immune thrombocytopenia, is a rare bleeding condition or a platelet disorder in which the immune system unintentionally attacks and destroys blood platelets, resulting in severe bruising and bleeding.

• Idiopathic: Because the reason for the immune reaction is unclear in about half of the cases, they are categorized as "idiopathic."
• Thrombocytopenia: Refers to a decline in thrombocyte (platelet) count.
• Purpura: Refers to purple regions of skin, mucous membranes, and the outer lining of organs where bleeding has occurred due to a low platelet count.

Two types of ITP include

1. Acute ITP
   1. Generally observed in young children, and both males and females are affected equally. This is the most common type of ITP.
   2. Symptoms of a viral infection are common but not always observed.
   3. Approximately 85 percent of patients heal within a year, and the disease does not reoccur.
2. Chronic ITP
   1. Symptoms can begin at any age and extend from six months to several years and even a lifetime.
   2. This type is more common in adults but can also occur in teens.
   3. Women develop it two to three times more often than men.
   4. Chronic ITP can reoccur and may need constant monitoring.

ITP can be characterized as acute or chronic, childhood or adult, and primary or secondary. The chronic type of ITP is defined by the disorder's continuation beyond 12 months.

A healthy platelet count ranges from 150,000 to 450,000 per microliter. The platelet count is fewer than 100,000 per microliter in patients with idiopathic thrombocytopenic purpura (ITP). When serious bleeding starts, the platelet count may be fewer than 10,000 per microliter. The risk of bleeding increases when the platelet count decreases. ITP symptoms are associated with increased bleeding because platelets stop bleeding. However, each person's symptoms may differ.

11 common signs and symptoms of ITP include

1. Easy or excessive bruising
2. Purple areas on the skin, often around the shins, causing a skin rash that looks like petechial rash (pinpoint red spots)
3. Hematoma or pooling of clotted or partially clotted blood under the skin; a hematoma may cause pain and swelling
4. Nosebleeds
5. Bleeding in the mouth or in and around the gums
6. Heavy menstrual periods
7. Fresh blood in vomit, urine or stool
8. Continuous bleeding post injury (As the patient lacks sufficient platelets to stop the bleeding, this is the most dangerous symptom and can be fatal.)
9. Internal bleeding
10. Fatigue
11. Low mood or depression

The above symptoms can be divided into three categories that include

1. Mild: Keep a note of them
   • Occasional nosebleeds that can be stopped by applying pressure
   • Blood blisters in the mouth
   • Small bruises
   • Petechiae (tiny red or purple dots on the skin)
2. Moderate: Discuss with a doctor
   • Nosebleeds that last longer than 15 minutes
   • Bleeding from the gums, lips, mouth, esophagus or intestines
   • Blood in the urine, stool or vomit
   • Large, new bruises (bigger than about two inches)
   • Lots of new petechiae
3. Severe: Need to visit a doctor or emergency department immediately
   • Continuous bleeding from the gums, lips, mouth or throat
   • Suspected internal bleeding (brain, lung, muscles, joints and other)
   • Many large, new bruises or petechiae

Patients with ITP might have a wide range of symptoms. Some patients will only encounter a few, whereas others may have severe and persistent symptoms. ITP symptoms might mimic other health issues. A proper evaluation by the doctor is always necessary to rule out other conditions.

Because the etiology was previously unknown, it was dubbed idiopathic thrombocytopenic purpura (ITP). However, it is now obvious that this disorder is caused by the immune system, which is why it is now known as immunological thrombocytopenia.

Platelets are destroyed by the immune system, which produces antibodies. The spleen eliminates the platelets, resulting in a low platelet count. Moreover, it is thought that the immune system influences platelet production.

10 Potential risk factors for ITP include

• Autoimmune diseases (rheumatoid arthritis, lupus)
  • People with rheumatoid arthritis, lupus, and antiphospholipid syndrome appear to be at high risk.
• Gender (ITP is more frequent in young women.)
• Viral infections (including cytomegalovirus, varicella-zoster virus, hepatitis C and HIV)
• Infection with Helicobacter pylori
• Medication
• Pregnancy
• Some types of cancer
• Immunodeficiency syndromes (conditions in which the immune system is weaker than normal)
• Evans syndrome (causes low platelets and low red blood cells)
• Side effects of certain medications

A few studies have reported clusters of ITP occurrence within families although it is uncertain if an autoimmune illness in the family might be a risk factor for ITP.

Each year, 50 to 150 new cases of ITP are reported, and about half of these cases are reported in children. ITP cases are increasing, as more people get routine blood tests that can identify a low platelet count.

A doctor will ask questions about the patient’s medical history and perform a physical exam. Depending on the symptoms and medical history, they may recommend:

Complete blood count

• Examines red and white blood cells, platelets (blood clotting cells), and, occasionally, reticulocytes (juvenile red blood cells).
• Comprises hemoglobin and hematocrit, as well as additional information about red blood cells.

Peripheral smear

• Blood is studied under a microscope. Blood cells are examined to see whether they appear normal.

Bone marrow aspiration

• Examines platelet production and rules out any aberrant cells that the marrow may be producing that might affect platelet counts.

Other lab tests may be performed to rule out possible underlying conditions. Idiopathic thrombocytopenic purpura (ITP) is typically diagnosed by isolating the root cause of bleeding. If there are no other reasons responsible for the symptoms, only then ITP diagnosis is undertaken.

The purpose of idiopathic thrombocytopenic purpura (ITP) treatment is to either cure active bleeding or limit the risk of bleeding. Treatment options and timing of treatment for ITP are determined by various factors. Most doctors agree that therapy should be the only option if a patient is not bleeding and their platelet count is below 30,000. Individuals with a platelet count of about 30,000 are unlikely to experience clinically substantial bleeding that would put them in danger. The best therapy for ITP will be determined by a variety of criteria, including the patient's age, platelet count, symptoms, medical history, career, and lifestyle.

Monitoring

• ITP is unique to each individual. In certain circumstances, the only therapy necessary is observation. Patients who have a bleed or an accident may require hospitalization and platelet transfusions.
• For others, low platelet counts caused by ITP might last years and need continuing therapy.

Corticosteroids

• Anti-inflammatory medications are used to treat a wide range of illnesses. They suppress immune system activity, which reduces the autoimmune reaction that kills the platelets in patients with ITP, allowing platelet count to increase.
• The corticosteroids indicated for ITP are taken orally. They are the first-line therapy for ITP, but long-term or recurrent usage can result in a variety of negative effects and is not advised.

Immunoglobulin

• Another first-line treatment option. It is a blood product that has a high concentration of antibodies and is administered intravenously.
• Antibodies are proteins produced by the immune system to combat infectious pathogens and other external invaders. Immunoglobulin infusions are used to treat immunodeficiency and disorders in which the immune system destroys healthy cells (such as ITP).
• Immunoglobulin is thought to control immunological responses. Several immunoglobulin products are available in the market. The forms of immunoglobulins suggested for short-term therapy of ITP include IVIG and anti-D immunoglobulin.

Immunosuppressant drugs

• Suppress the immune system. They are precisely medications that interfere with immune system functioning. They are used to treat various medical illnesses.
• Although not licensed for the treatment of ITP, several immunosuppressant medications have been administered with promising outcomes.
• Immunosuppressive medications increase the risk of infections and several forms of cancer, and they are not safe to use during pregnancy.

Other medications: Several drugs are used to treat ITP. When other therapies have failed, they are used as a second or third line of treatment:

• Chemotherapy medications, which are commonly used to treat cancer, can also be used to treat ITP due to their capacity to control cell formation.
• Spleen tyrosine kinase inhibitors prevent antibodies from destroying platelets by inhibiting a protein.
• Thrombopoietin receptor agonists, which increase platelet synthesis in the bone marrow, may also be recommended.

Surgery

• Another therapeutic option for ITP is splenectomy (surgery to remove the spleen).
• Platelet-attacking antibodies are created in the spleen. If corticosteroids did not work, this was the go-to therapy in the past few years. It has shown to be a beneficial therapeutic choice for many people but not all.
• Today, with more treatment choices and new treatment alternatives being researched, it is considered a third-line therapy that is used after previous therapies have failed to treat ITP.
• Splenectomy is connected with various side effects. Living without a spleen puts a person at an increased risk of deadly infections and blood clots. When platelet levels are low, surgery also becomes a risk to consider.

Dietary guidelines for patients with ITP

Recommended foods

• Green leafy vegetables: Coriander, spinach, kale, fenugreek, etc.
• Should be part of the diet at least two times a week: Bitter gourd, ridge gourd, bottle gourd, cabbage, carrot, beetroot, pumpkin, and cauliflower
• A variety of fruits should be part of a healthy diet: Papaya, apple, kiwi, peach, pear, pomegranate, etc.
• Foods that include whole grain
• Vitamin K-rich foods to improve blood clotting
• Liquids: Goat milk, wheatgrass, aloe vera juice, pomegranate juice, and coconut water
• Papaya fruit and its leaves juice are beneficial for patients with ITP

Foods that should be avoided include

• Citrus fruits
• Alcohol
• Sweetened drinks
• Milk and milk products
• Processed and fried food
• Spices and spicy food items
• Excessive salt and red chilies
• Nonvegetarian diet but the patient can have only two boiled egg white

ITP treatment will be determined by the severity of the patient’s condition.

The life expectancy of patients with idiopathic thrombocytopenic purpura (ITP) is determined by the type of disease, symptoms, and age. Most patients with ITP have a good outcome, with few hospitalizations and fewer chances of mortality. Almost all patients can live completely normal life. It has been demonstrated that 80 percent of children diagnosed with ITP recover completely after six months, regardless of treatment. The younger the patient, the lower their risk of chronic ITP.

Although it is possible that ITP has no effect on a person's way of life, it is still recommended to take certain precautions in the event of any symptoms (bleeding), as well as to visit a doctor frequently. Patients with persistent severe thrombocytopenia who do not respond to therapy within the first two years face significant morbidity and mortality.

• The majority of people with ITP do not have a serious or life-threatening condition. Without treatment, acute ITP in children usually resolves in six months or less.
• ITP can be chronic for many years. Even in severe cases, people can live for decades with the disease.

Idiopathic thrombocytopenic purpura (ITP), while related to other viruses, is uncommon in conjunction with coronaviruses. During the first SARS coronavirus outbreak, the following factors were found to be responsible for ITP:

• Numerous antibiotics and antivirals
• Heparin
• Hemodialysis
• Extracorporeal membrane oxygenation

Although the causes of thrombocytopenia, both immune and nonimmune, in COVID-19 are still unclear, they are thought to be comparable to the processes found in SARS-CoV-1 infection:

• Decreased platelet production caused by the cytokine storm
• Infection of bone marrow stromal cells and hematopoietic stem cells
• Increased platelet destruction as a result of autoreactive antibodies
• Formation of immune complexes
• Decreased circulating platelet count due to lung injury

The fact that steroids were beneficial in restoring platelet counts in these few case studies suggests that the inflammatory cytokine storm had a substantial role in triggering thrombocytopenia because these steroids would have helped attenuate the effects of the cytokine release. ITP is uncommon in SARS-CoV-2 infection, but it serves as a marker of potential "side effects" of infection and adds a new dimension to consider when deciding the optimal course of therapy for a specific patient.

A possible relationship between COVID-19 vaccinations and idiopathic thrombocytopenic purpura (ITP) is being explored. This is due to the recognized association between measles, mumps, and rubella vaccination and ITP, as well as the fact that COVID-19 can induce ITP. The effect of COVID-19 vaccination on preexisting ITP (acute and chronic) is not well understood. Limited and preliminary evidence suggests that vaccination may aggravate thrombocytopenia in 10 percent of people with chronic ITP after vaccination.

It is crucial to highlight, however, that ITP is most typically triggered by a virus and that the risk of recurrence or worsening of ITP is likely to be greater if these individuals develop COVID-19 than the danger of vaccination itself. Vaccination is thus suggested for patients with a history of ITP.

There is still a chance of recurrence regardless of vaccination brand; however, this risk is high with COVID-19 itself. Following vaccination, close monitoring should is recommended to ensure that there is no additional reduction in platelets.

Monitoring of platelets and possible therapy escalation may be necessary if clinical symptoms worsen (days to weeks after vaccination) due to the vaccine. After receiving COVID-19 vaccines, patients who experience ITP can receive additional doses (including booster doses) as soon as it is deemed safe to do so.

It is advised to postpone vaccination until platelet counts are stable. A patient should consult a hematologist about their options if they are still receiving immunosuppressive medications such as corticosteroids.