Thrombocytopenia

Symptoms Of Thrombocytopenia

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

• Getting bruised easily 

• Prolonged/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 are called 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. This can cause symptoms like headaches and other neurological signs. 

Low platelet count can also occur due to reasons such as pregnancy. Such cases usually don't result in any symptoms and may go undetected. 

Severe cases can result in excessive and uncontrollable bleeding from wounds. These patients require immediate medical attention as continued bleeding might result in hemorrhagic shock or hypovolemic shock which may prove fatal, if left untreated.  

Causes Of Thrombocytopenia

The normal range of platelet count is from 150,000 to 400,000 per microliter. Thrombocytopenia means 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 the following: 

1. Increased platelet destruction

2. Decreased platelet production

3. Increased sequestration or trapping of platelets

4. 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 starts to attack healthy cells of the body. One example of this type of autoimmune disease is immune thrombocytopenia (ITP). In ITP, the immune system attacks and destroys its own platelets. The exact cause of this condition is not known. Other autoimmune diseases such as antiphospholipid syndrome, systemic lupus erythematosus, rheumatoid arthritis and sarcoidosis can also cause thrombocytopenia.

• Medications: Certain medications such as heparin (anticoagulant medication that prevents clot formation), quinine and valproic acid can sometimes trigger the immune system and result in the formation of anti-platelet antibodies that attack the body's own platelets. Sulfa containing antibiotics and anticonvulsants are some other medications that can cause thrombocytopenia. 

• Infections: Bacterial infections such as Helicobacter pylori infections, leptospirosis, brucellosis and anaplasmosis can result in low platelet count. Chronic viral infections like hepatitis C and HIV anti-platelets antibodies are present which cause platelet destruction.

• Surgery: Platelets can get destroyed when they pass through artificial heart valves, blood vessel grafts, or machines and tubes used for blood transfusions or bypass surgery.

• Pregnancy: During pregnancy, some women develop a mild to moderate form of thrombocytopenia when they are close to delivering a baby. Thrombocytopenia during the third trimester is usually mild and resolves soon after delivery. Doctors may continue to monitor platelet count through the remaining pregnancy to prevent the number from dropping further. 

• Thrombotic thrombocytopenic purpura (TTP): It is a rare blood disorder in which blood clots start developing in small blood vessels in the body. These clots reduce the number of circulating platelets and result in thrombocytopenia. 

• Disseminated intravascular coagulation (DIC): It is a rare complication of pregnancy, severe infections, or severe trauma. In this condition, the blood clots form suddenly throughout the body that use up many of the blood's platelets.

• Hemolysis, elevated liver tests, low platelets (HELLP) syndrome: It is another non-immune thrombocytopenia that may occur during pregnancy.

• Hemolytic uremic syndrome: It is a rare disorder caused by shiga toxin-producing organisms (E. coli and Shigella) that leads to a decline in the number of platelets.

2. Conditions that cause decreased platelet production

There are some conditions in which the bone marrow fails to produce enough platelets. The causes include:

• Cancers: Leukaemia (blood cancer), lymphoma and myelodysplastic syndromes cause uncontrollable growth of abnormal cells in the bone marrow. These abnormal cells crowd out the healthy cells that are typically present in the bone marrow, including platelets. Thrombocytopenia is a widespread and early presentation in patients with acute leukaemia. Patients who have cancer can also experience low platelet count as a side effect of chemotherapy.

• Bone marrow failure: It is seen in aplastic anemia and paroxysmal nocturnal hemoglobinuria which can cause severe reduction in platelet count. Aplastic anemia is a rare condition in which the body stops producing new blood cells. Such individuals often develop a low platelet count and deficiency of red and white blood cells. Paroxysmal nocturnal hemoglobinuria is closely related to aplastic anemia.

• Chronic alcoholism: Thrombocytopenia is commonly seen in heavy drinkers since alcohol exposure slows down the production of megakaryocytes, large bone marrow cells that are responsible for producing platelets. 

• Viral infections: Infections caused by varicella-zoster virus, cytomegalovirus, epstein-barr virus, mumps virus, parvovirus B19, rickettsia, rubella virus, zika virus, hepatitis C virus and HIV can cause reduced platelet production.

• Nutritional deficiencies: Lack of Vitamin B12, folate and copper can cause low platelet production.

• Toxic chemicals: Exposure to toxic chemicals such as arsenic, benzene, and pesticides can affect the normal production of platelets.

• Cirrhosis or scarring of liver: It causes reduced production of thrombopoietin, a hormone which regulates the production of platelets. This leads to reduced production of platelets by the bone marrow.

• 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 syndromes, and Shwachman-Diamond syndrome.

• Medications: Over-the-counter medicine such as aspirin or ibuprofen can affect production of platelets. Certain medicines like diuretics, chloramphenicol, methotrexate, interferon, carboplatin, and amiodarone, also cause bone marrow to decrease production of platelets.

3. Conditions that cause increased trapping of platelets

Enlarged spleen can also lead to thrombocytopenia. The spleen is a vital organ that fights off infections and plays a role in filtering any unwanted substances from your blood. Normally, one-third of platelet mass is in the spleen.

The spleen can get enlarged due to medical conditions such as cirrhosis of the liver, a late-stage liver disease that causes permanent damage to the liver. It is also seen in Gaucher's disease, an inherited condition that causes fatty substances to build up in tissues and bone marrow conditions, such as myelofibrosis.

In such cases, the enlarged spleen traps platelets and prevents their circulation into the bloodstream. This results in sequestration (removal) of platelets up to 90% of the spleen's total mass, resulting in thrombocytopenia.

4. Conditions that cause dilution of blood

Dilution of blood in cases of massive blood transfusion and massive fluid resuscitation can cause thrombocytopenia.

Risk Factors For Thrombocytopenia

In adults, women are more likely to develop thrombocytopenia than men. Individuals who are at highest risk for thrombocytopenia are those affected by one of the conditions discussed in causes of thrombocytopenia. These people 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 have reactions to certain medications 

• Long term heavy alcohol drinkers

• Pregnant women

Diagnosis Of Thrombocytopenia

History

Obtaining a thorough history helps to identify the cause of thrombocytopenia. The following questions will help your doctor determine the cause and frame a suitable treatment plan.

• History of bleeding

• Prior blood count testing and baseline platelet count and the 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, and 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 or whether a patient is taking over-the-counter medications, quinine-containing beverages, or herbal teas

• In the hospitalised patient, 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 assesed

Physical examination 

If your healthcare provider suspects that you have thrombocytopenia, they will start with carrying out a physical examination. 

• You will be checked for any signs of bleeding caused by thrombocytopenia which presents as red or purple flat discoloured spots on the skin

• Your doctor will also feel your abdomen to check for signs of enlarged spleen and liver

• Examination of any enlarged lymph nodes 

Blood tests

Complete blood count (CBC) test: The CBC test is a diagnostic tool that evaluates the number of blood cells in your blood. It will allow the doctor to know if you have lower than the average platelet count. Usually, the platelet count of healthy people falls in the range of 150,000 to 450,000 platelets per mL of blood. Platelet indices like mean platelet volume (MPV) and platelet distribution width (PDW) are evaluated to diagnose thrombocytopenia.

Blood smear test: A blood smear test requires a small sample of your blood that is examined under a microscope to check the appearance of your platelets under the microscope.

Platelet antibodies blood test: Your body may be creating antibodies that are attacking platelets instead of infections. To evaluate the presence of platelet antibodies, your doctor may order a platelet antibodies test. This test is often ordered if you present with a bleeding problem. Platelet antibodies can be produced as a side effect of certain drugs such as quinine or other reasons.

Blood coagulation profile: Your doctor may order blood clotting tests such as prothrombin time and partial thromboplastin test. It is a simple blood test that requires a sample of your blood into which some reagent chemicals are added to test the number of seconds it takes for a clot to form.  

Other tests: Additional blood test that can help to evaluate the cause of thrombocytopenia include:

• Liver function tests 
• Kidney function tests 
• Vitamin B12 levels 
• Folic acid levels
• Copper levels

Abdomen ultrasound

In case your doctor suspects that you have an enlarged spleen, you may be asked to get an ultrasound. This test is a diagnostic tool that uses sound waves to visualise your spleen. It can help your doctor determine the size of your spleen.

Bone marrow aspiration and biopsy 

The bone marrow aspiration test is done, if you present signs of a disorder that affects your bone marrow. During the bone marrow aspiration test, a needle is used to remove a small amount of fluid from the bone marrow of one of the bones. A clinician will examine the sample under the microscope to check for abnormal cells. A bone marrow biopsy requires the removal of a small sample of bone marrow tissue from the bone using a needle. 

Watch this video to know about the complete blood count test & its implications.

Prevention Of Thrombocytopenia

Prevention of thrombocytopenia depends on the cause behind the condition. Usually, you can't prevent a low platelet count, but if you are at risk of developing a low platelet count due to an underlying condition or medical history, you should take prevention measures like:

• You can try avoiding heavy drinking since alcohol reduces the development of platelets. 

• Avoid contact with toxic chemicals, including arsenic and benzene.

• If you are taking medications that are known to lower platelet count, talk to your doctor about alternative drugs. Medicines like aspirin and ibuprofen tend to thin the blood and may increase your chances of excessive bleeding.

• Viral infections are known to reduce platelet count. Practice basic hand hygiene and wash your hands before eating. 

• You can also talk to your doctor about getting vaccinated against diseases like chickenpox, measles, mumps and rubella. You should have your children vaccinated for these viruses as well. Talk to your child's pediatrician about these vaccines.

• You should avoid activities with a high risk of bleeding or bruising like contact sports.

Specialist To Visit

You should visit a doctor, if you are experiencing symptoms such as quickly getting bruises, reddish or purplish spots on your lower legs, blood in your urine or stool, excessive bleeding from nose or mouth, abnormally excessive bleeding during menstruation, headache, and other neurological symptoms. These symptoms might indicate the possibility of a low platelet count. You can consult the following doctors for diagnosis:

• General physician

• Hematologist

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Treatment Of Thrombocytopenia

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. 

In case 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 

1. 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. 

2. 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. 

3. 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. 

4. 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 a long-term. Examples include:

• Eltrombopag

• Romiplostim 

• Avatrombopag 

5. Fostamatinib is a newer drug used for the treatment of chronic immune thrombocytopenia and those 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 since 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/ul 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/ul in an adult of 70 kg weight. Single unit 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. 

Home-care For Thrombocytopenia

• If you have been prescribed medications to manage low platelet count, make sure you take those medications on time. Label your drugs and set the alarm to make sure you have the medicines every day at the same time. Follow all the instructions given to you by your doctor. 

• Some instructions provided by your healthcare provider may include regularly examining your skin for any scrapes, cuts or bruises. 

• Use an electric shaver and a soft toothbrush or mouthwash to avoid injuring your face or mouth. 

• Ensure that your nails are trimmed to avoid accidental scrapes. 

• If you tend to get constipated, ask your provider for some laxatives to help you pass a motion without strain. Straining while passing motion can cause bleeding in the brain due to increased pressure.

• If you have just had a splenectomy procedure, follow post-op instructions given by your doctor closely and monitor your health. 

Note: People with low platelet counts are more likely to have bleeding of the nose. In that case, it is advisable to lean ahead and bend forward to prevent clogging of the mouth and throat with blood. Read about the other possible causes and first-aid tips to manage nose bleeds.
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Complications Of Thrombocytopenia

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

• Spotting blood in urine

• Dark or tarry, red coloured stools 

• Severe headache

• Other neurological symptoms (in case of bleeding in the brain)

• Hemorrhagic shock

Patients who are advised to undergo splenectomy are given different vaccines to help them prevent infection. Spleen in a part of the immune system and removal of the organ leaves a patient more susceptible to catching infections. Formation of blood clots and bleeding are some other complications of splenectomy. 

Alternative Therapies For Thrombocytopenia

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

Vitamin C

Vitamin C helps in the grouping of platelets and increases their functional efficiency. It also helps in the absorption of iron and increases the platelet count. Broccoli, tomatoes, cauliflower, mangoes and pineapple are good sources of Vitamin C. 

Iron 

Iron plays a vital role in the production of healthy blood cells. Iron supplements can increase platelet count in patients with iron deficiency anaemia. Mussels, lentils, beef, and pumpkin seeds are excellent sources of iron. 

Papaya leaf extract 

Papaya leaf extract can noticeably improve the 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 when they took papaya leaf juice. 

Melatonin 

The body naturally produces melatonin, and it primarily helps in improving sleep quality. It is also available in the form of tablets, liquid or lotions. Melatonin has been found to increase platelet levels in patients with thrombocytopenia. 

Vitamin B12 

It's been reported that a deficiency of Vitamin B12 is linked with low platelets count. Apart from that, vitamin B12 also helps in maintaining the health of blood cells. Food sources such as clams, eggs, and beef liver are rich in Vitamin B12. Along with animal-based products, dairy products are also rich sources of Vitamin B12. However, in some cases, cow milk can interfere with the production of platelets.  

Living With Thrombocytopenia

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 and 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 getting involved in any physical activity that can cause your injuries. Consult your doctor about the activities that are safe for your conditions. Often 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 of reducing your risk of developing a severely low platelet count. 

Exercise caution with OTC medications

We often tend to self medicate with aspirin or ibuprofen when we have pain. However, in patients with a low platelet count, these medications can further decrease the number of platelets and prevent the platelets from working efficiently. Consult your doctor about the medicines you can take safely that won't adversely affect your platelet count. 

Use a humidifier 

Using a cool-mist humidifier in the home can increase its moisture content. This can further prevent nosebleeds or coughs. Excessive coughing tends to put more pressure on your brain, which can further increase your risk of internal bleeding in patients with 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 you get the proper medical treatment in adverse situations.