Thrombocytopenia

Thrombocytopenia can cause several symptoms that affect your daily life. It is possible to experience more than one symptom at a time.

• Getting bruised easily
• Prolonged or excessive bleeding from wounds
• Superficial bleeding that appears as pinpoint red spots called petechiae
• Bleeding from gums or nose
• Enlarged spleen
• Extreme fatigue
• Blood in urine or stool
• Heavy menstrual flow
• Bleeding inside the gastrointestinal (stomach) tract
• Bleeding from the anus
• Unusual bleeding after dental work or surgery
• Red, brown, or purple bruises known as purpura

You need to talk to your doctor immediately if you observe signs of internal bleeding, such as blood in your vomit, stool, or urine. This condition can rarely lead to bleeding in the brain when a patient's platelet count becomes excessively low, causing symptoms like headaches and other neurological signs. Low platelet counts can also occur due to reasons such as pregnancy, which usually do not result in any symptoms and may go undetected. Severe cases can lead to excessive and uncontrollable bleeding from wounds, requiring immediate medical attention as continued bleeding might result in hemorrhagic shock or hypovolemic shock, which can be fatal if left untreated.

The normal range of platelet count is from 150,000 to 400,000 per microliter. Thrombocytopenia indicates that your body has fewer than the average number of platelets per microliter of circulating blood. Circulating platelets can be reduced for one or more reasons, which include:

• Increased platelet destruction
• Decreased platelet production
• Increased sequestration or trapping of platelets
• Dilution of blood

1. Conditions that cause increased destruction of platelets

The bone marrow is responsible for producing platelets in the body. In some specific conditions, the immune system starts producing antibodies that attack platelets instead of infections. Some causes include:

• Autoimmune diseases: In autoimmune diseases, the body's immune system attacks healthy cells. An example is immune thrombocytopenia (ITP), where the immune system destroys its own platelets. Other autoimmune diseases such as antiphospholipid syndrome, systemic lupus erythematosus, rheumatoid arthritis, and sarcoidosis can also cause thrombocytopenia.
• Medications: Certain medications, such as heparin, quinine, and valproic acid, can trigger the immune system to form anti-platelet antibodies. Sulfa-containing antibiotics and anticonvulsants are other medications that can cause thrombocytopenia.
• Infections: Bacterial infections like Helicobacter pylori, leptospirosis, brucellosis, and anaplasmosis can lead to low platelet count. Chronic viral infections such as hepatitis C and HIV can produce anti-platelet antibodies, causing platelet destruction.
• Surgery: Platelets can be destroyed when they pass through artificial heart valves, blood vessel grafts, or machines and tubes used for blood transfusions or bypass surgery.
• Pregnancy: Some women develop mild to moderate thrombocytopenia close to delivery. This condition usually resolves soon after childbirth, but doctors may monitor platelet count to prevent further drops.
• Thrombotic thrombocytopenic purpura (TTP): A rare blood disorder where blood clots form in small blood vessels, reducing the number of circulating platelets.
• Disseminated intravascular coagulation (DIC): A rare complication of pregnancy, severe infections, or trauma, where sudden blood clots use up many platelets.
• Hemolysis, elevated liver tests, low platelets (HELLP) syndrome: A non-immune thrombocytopenia that may occur during pregnancy.
• Hemolytic uremic syndrome: A rare disorder caused by shiga toxin-producing organisms (E. coli and Shigella) that leads to a decline in platelet count.

2. Conditions that cause decreased platelet production

Some conditions cause the bone marrow to fail in producing enough platelets. The causes include:

• Cancers: Leukemia, lymphoma, and myelodysplastic syndromes lead to the uncontrollable growth of abnormal cells in the bone marrow, crowding out healthy cells, including platelets. Thrombocytopenia is an early presentation in patients with acute leukemia.
• Bone marrow failure: Seen in aplastic anemia and paroxysmal nocturnal hemoglobinuria, which can cause severe reduction in platelet count. Aplastic anemia is a rare condition where the body stops producing new blood cells.
• Chronic alcoholism: Thrombocytopenia is commonly seen in heavy drinkers due to slowed production of megakaryocytes, the large bone marrow cells responsible for producing platelets.
• Viral infections: Infections from varicella-zoster virus, cytomegalovirus, Epstein-Barr virus, mumps virus, parvovirus B19, rickettsia, rubella virus, zika virus, hepatitis C virus, and HIV can reduce platelet production.
• Nutritional deficiencies: Lack of Vitamin B12, folate, and copper can lead to low platelet production.
• Toxic chemicals: Exposure to toxic chemicals such as arsenic, benzene, and pesticides can impair normal platelet production.
• Cirrhosis or scarring of the liver: This condition reduces the production of thrombopoietin, a hormone that regulates platelet production.
• Congenital conditions: Conditions like Fanconi anemia, gray platelet syndrome, Wiskott-Aldrich syndrome, Alport syndrome, Bernard-Soulier syndrome, platelet-type or pseudo-von Willebrand disease, May-Hegglin syndrome, and Shwachman-Diamond syndrome.
• Medications: Over-the-counter medications like aspirin or ibuprofen can affect platelet production. Certain medications such as diuretics, chloramphenicol, methotrexate, interferon, carboplatin, and amiodarone can also decrease platelet production.

3. Conditions that cause increased trapping of platelets

An enlarged spleen can lead to thrombocytopenia. The spleen is vital for fighting infections and filtering unwanted substances from the blood. Normally, one-third of platelet mass is in the spleen. Conditions such as liver cirrhosis, Gaucher's disease, and myelofibrosis can cause spleen enlargement. This enlargement traps platelets and prevents their circulation into the bloodstream, resulting in sequestration of platelets up to 90% of the spleen's total mass.

4. Conditions that cause dilution of blood

Dilution of blood due to massive blood transfusions and fluid resuscitation can cause thrombocytopenia.

In adults, women are more likely to develop thrombocytopenia than men. Individuals at the highest risk for thrombocytopenia are those affected by one of the conditions discussed in the causes of thrombocytopenia. These individuals include:

• Having a family history of immune thrombocytopenia (ITP)
• Suffering from autoimmune disorders such as lupus or rheumatoid arthritis
• Suffering from cancer or being exposed to radiation treatments or chemotherapy
• Being exposed to toxic chemicals or having reactions to certain medications
• Long-term heavy alcohol drinkers
• Pregnant women

History

• History of bleeding
• Prior blood count testing, baseline platelet count, and recent drop in platelet count
• Any potential exposure and symptoms of viral, bacterial, or rickettsial infections
• Assessment of risk factors for HIV infection
• Travel to an area endemic for malaria, dengue, or Ebola
• Diet history to detect any nutritional deficiencies of Vitamin B12, folic acid, or copper
• Check for other conditions like systemic lupus erythematosus or rheumatoid arthritis
• History of any bariatric surgery or blood transfusion
• Review of medications taken, including over-the-counter medications, quinine-containing beverages, or herbal teas
• In hospitalized patients, assessment for exposure to heparin products
• Check for a family history of thrombocytopenia or bleeding disorders
• In pregnant women, history of headache, visual symptoms, abdominal pain, or flu-like symptoms is assessed

Physical Examination

• Check for signs of bleeding caused by thrombocytopenia, which may present as red or purple flat discolored spots on the skin
• Palpation of the abdomen to check for signs of enlarged spleen and liver
• Examination of any enlarged lymph nodes

Blood Tests

• Complete blood count (CBC) test: This diagnostic tool evaluates the number of blood cells in your blood, allowing the doctor to determine if you have a lower than average platelet count. The normal platelet count ranges from 150,000 to 450,000 platelets per mL of blood. Platelet indices like mean platelet volume (MPV) and platelet distribution width (PDW) are also evaluated.
• Blood smear test: This test requires a small sample of your blood, which is examined under a microscope to check the appearance of your platelets.
• Platelet antibodies blood test: This test evaluates the presence of antibodies that may be attacking your platelets. It is often ordered if you present with a bleeding problem and can be a side effect of certain drugs such as quinine.
• Blood coagulation profile: Tests such as prothrombin time and partial thromboplastin time are conducted to evaluate blood clotting. This simple blood test measures the time it takes for a clot to form after adding reagent chemicals to a blood sample.
• Other tests: Additional blood tests that can help evaluate the cause of thrombocytopenia include:
  • Liver function tests
  • Kidney function tests
  • Vitamin B12 levels
  • Folic acid levels
  • Copper levels

Abdomen Ultrasound

Bone Marrow Aspiration and Biopsy

Prevention of thrombocytopenia depends on the underlying cause of the condition. While you typically cannot prevent a low platelet count, if you are at risk due to a medical history or condition, you should consider the following preventive measures:

• Avoid heavy drinking, as alcohol can inhibit platelet development.
• Steer clear of toxic chemicals, including arsenic and benzene.
• If you are on medications known to lower platelet counts, consult your doctor about alternative options. Medications such as aspirin and ibuprofen can thin the blood and increase the risk of excessive bleeding.
• Practice good hand hygiene to reduce the risk of viral infections, which can lower platelet counts. Wash your hands before eating.
• Discuss vaccination with your doctor for diseases such as chickenpox, measles, mumps, and rubella. Ensure your children are vaccinated against these viruses as well; consult your child's pediatrician about the vaccines.
• Avoid activities with a high risk of bleeding or bruising, such as contact sports.

The treatment plan for low platelet count depends on the cause of thrombocytopenia and the severity of the disorder. Doctors decide the course of action with the primary goal of preventing any disability or loss of life due to thrombocytopenia. Generally, mild cases of low platelet count go undiagnosed and may not require any specific treatment. In such cases, your doctor may hold off treatment and simply monitor you for some time. Mild low platelet count also tends to improve when the underlying cause behind the condition is treated. If you have a low platelet count because of an infection, the resultant thrombocytopenia will resolve once the disease is treated. If you have a low platelet count as a result of an adverse reaction to a drug, your doctor will switch to alternative medicines. Generally, drug-induced low platelet count resolves after the responsible medication is stopped. Thrombocytopenia due to an immune system condition can be resolved by taking medicines that suppress the immune system.

A) Medications

• Corticosteroids like dexamethasone or prednisone are often prescribed to raise platelet count. Steroids tend to reduce the rate of platelet destruction and can be administered orally or through the veins.
• IV immunoglobulins (IVIG) are used for treatment of thrombocytopenia in case you cannot tolerate steroids or if your platelet count drops after completion of steroid treatment.
• Rituximab helps suppress the immune system to prevent the immune system from attacking its own platelets. This is often prescribed to patients who have persistent ITP.
• Thrombopoietin (TPO) receptor agonists, also known as platelet growth factors, might be recommended if treatment with steroids, splenectomy, or rituximab fails to increase the platelet count. These medicines might be prescribed for long-term use. Examples include:
  • Eltrombopag
  • Romiplostim
  • Avatrombopag
• Fostamatinib is a newer drug used for the treatment of chronic immune thrombocytopenia in patients who haven't responded to other treatments.

B) Blood or Platelet Transfusion

Patients who are at a high risk of bleeding or are actively bleeding are treated with blood or platelet transfusions. A needle is inserted to introduce an intravenous line in your body through which you will receive healthy platelets or blood. This procedure is advised for patients with extremely low platelet count, as transfused platelets can last only for around three days in circulation. This is a temporary treatment that transiently raises your platelet count. There are two options for platelet transfusion:

• Random donor platelet concentrates (RDPC): RDP is a platelet transfusion method in which platelets are prepared by centrifuging the whole blood collected from four to five donors and pooling the platelets. With the help of this method, the platelet count is elevated to 5000-10000/μL in an adult of approximately 70 kg body weight.
• Single donor platelet concentrates (SDPC): SDP is a platelet transfusion method in which platelets are prepared from a single donor by an apheresis machine. A unit of platelet concentrate prepared by this method increases the platelet count by 30000-70000/μL in an adult of 70 kg weight. A single unit of SDPC has a platelet content similar to that of 6-8 units of pooled platelets or RDPCs.

C) Splenectomy

Splenectomy is a surgical procedure in which the spleen is removed from the body. This procedure is usually the last line of treatment and is advised when medications fail to treat low platelet count effectively. It is generally recommended for patients who have immune thrombocytopenia. Once the spleen is removed, a low platelet count resolves in more than half of patients who have ITP.

If you have been prescribed medications to manage low platelet count, ensure you take those medications on time. Label your drugs and set an alarm to remind you to take your medicines daily at the same time. Follow all instructions provided by your doctor.

Some instructions provided by your healthcare provider may include:

• Regularly examining your skin for any scrapes, cuts, or bruises.
• Using an electric shaver and a soft toothbrush or mouthwash to avoid injuring your face or mouth.
• Ensuring that your nails are trimmed to prevent accidental scrapes.
• If you tend to get constipated, ask your provider for laxatives to help you pass a motion without strain, as straining can lead to bleeding in the brain due to increased pressure.
• If you have recently undergone a splenectomy, closely follow post-operative instructions from your doctor and monitor your health.

Note: Individuals with low platelet counts are more susceptible to nosebleeds. In such cases, it is advisable to lean forward to prevent blood from clogging the mouth and throat. Read about other possible causes and first-aid tips to manage nosebleeds.

People with severe thrombocytopenia are at an increased risk of internal bleeding into the intestines or the brain. Internal bleeding can be fatal and requires immediate medical attention. Signs that may indicate internal bleeding include:

• Spotting blood in urine
• Dark or tarry, red-colored stools
• Severe headache
• Other neurological symptoms (in case of bleeding in the brain)
• Hemorrhagic shock

Patients advised to undergo splenectomy are given various vaccines to help prevent infection. The spleen is part of the immune system, and removal of this organ leaves a patient more susceptible to infections. Formation of blood clots and bleeding are additional complications associated with splenectomy.

If you have mild or moderate thrombocytopenia, you can eat certain foods and supplements to raise your platelet count. Severely low platelet count, however, requires medical treatment. Always consult your doctor before taking any supplement to prevent interactions with other medications.

Vitamin C

Vitamin C helps in the grouping of platelets and increases their functional efficiency. It also aids in the absorption of iron and boosts platelet count. Good sources of Vitamin C include:

• Broccoli
• Tomatoes
• Cauliflower
• Mangoes
• Pineapple

Iron

Iron plays a vital role in the production of healthy blood cells. Iron supplements can increase platelet count in patients with iron deficiency anemia. Excellent sources of iron include:

• Mussels
• Lentils
• Beef
• Pumpkin seeds

Papaya Leaf Extract

Papaya leaf extract can noticeably improve platelet count in animals. You can consume papaya leaf extract in the form of pills. A study reported increased platelet count in patients with dengue fever who took papaya leaf juice.

Melatonin

The body naturally produces melatonin, which primarily helps improve sleep quality. It is also available in tablet, liquid, or lotion form. Melatonin has been found to increase platelet levels in patients with thrombocytopenia.

Vitamin B12

A deficiency of Vitamin B12 is linked with low platelet counts. Additionally, Vitamin B12 helps maintain the health of blood cells. Food sources rich in Vitamin B12 include:

• Clams
• Eggs
• Beef liver
• Dairy products

However, in some cases, cow's milk can interfere with the production of platelets.

If you have been diagnosed with a mild to moderate level of thrombocytopenia, you may not need any treatment. Constant monitoring and follow-ups as requested by your doctor can be enough to manage the condition. Low platelet count due to medical conditions tends to resolve after the disease is treated. Patients with severe cases of thrombocytopenia may need treatment. Your doctor will work with you to devise a treatment plan that will help you manage your symptoms.

Check for bruises and scrapes:

• Always remember to check your body for any signs of bruises or scrapes after engaging in physical activities that can cause injuries.
• Consult your doctor about the activities that are safe for your condition.
• Contact sports such as football, boxing, or martial arts carry a high risk of physical injury, leading to uncontrollable bleeding in cases of severe thrombocytopenia.

Drink alcohol in moderation:

• Alcohol slows down the production of platelets in the body.
• If you have thrombocytopenia, consult your doctor about how much alcohol you can safely consume.
• Avoiding alcohol is an excellent way to reduce your risk of developing a severely low platelet count.

Exercise caution with OTC medications:

• Aspirin and ibuprofen are commonly used for pain relief, but in patients with a low platelet count, these medications can further decrease platelet numbers and impair their function.
• Consult your doctor about safe medications that won't adversely affect your platelet count.

Use a humidifier:

• Using a cool-mist humidifier in the home can increase moisture content and help prevent nosebleeds or coughs.
• Excessive coughing can put more pressure on your brain, increasing the risk of internal bleeding in patients with a low platelet count.

Wear a medical alert identification:

• Wearing a medical alert bracelet or carrying a card stating that you have a low platelet count can help ensure you receive proper medical treatment in emergencies.