Introduction
Platelets, also known as thrombocytes, are blood cells that form in the bone marrow. Normally, when one of the blood vessels is injured, one starts to bleed. The platelets will clot (clump together) to form a plug in the blood vessel and stop the bleeding. The platelet count is about one lakh forty thousand to four lakh forty thousand per microliter. The platelet count can vary slightly according to the menstrual cycle phase; it decreases during near-term pregnancy (known as gestational thrombocytopenia) and increases in response to inflammatory cytokines (known as secondary or reactive thrombocytosis).
Thrombopoietin stimulates the bone marrow to produce mega keratocytes, which control the platelets in circulation and is produced in the liver at a fixed rate, and its movement is determined by the extent to which moving platelets are cleared. Platelets circulate for seven to ten days in the bloodstream. About one-third are always transiently sequestered in the spleen; the process is called apoptosis.
What Is a Platelet Disorder?
Platelet disorders often mean that injured blood vessels bleed heavily and heal rather slowly.
What Are the Types of Platelet Disorders?
There are different groups of disorders affecting the platelets:
A) Thrombocythemia and Thrombocytosis - Overproduction of platelets because of a clonal abnormality of a hematopoietic stem cell. The platelet count and risk of thrombosis are not correlated, but some patients with high thrombocytosis count (i.e., > 1,000,000/mcL) develop bleeding due to loss of high molecular weight.
B) Reactive Thrombocytosis - It is the overproduction of platelets in response to some underlying disorder. The causes could be an acute infection, chronic inflammatory disorders (e.g., rheumatoid arthritis, tuberculosis), iron deficiency, and cancers (some).
C) Thrombocytopenia - It results in a reduction of platelets, causing a risk for mild or serious bleeding. The causes include:
-
Platelet production is reduced.
-
The number of platelet destruction in the spleen is increased in comparison to the overall production.
-
Consumption of platelets is more than the production.
-
Weakened or diluted platelets.
Many drugs may also cause thrombocytopenia, usually by triggering immunologic destruction. The most common specific causes of thrombocytopenia include:
-
Pregnancy (called gestational thrombocytopenia; HELLP syndrome [hemolysis, elevated liver enzymes, and low platelets]).
-
Drugs that cause changes in the immune system, resulting in platelet destruction (namely, Heparin, Trimethoprim/Sulfamethoxazole, rarely Quinine [cocktail purpura] or Abciximab).
-
Drugs that are used as bone marrow suppressants whose actions are dose-dependent (such as chemotherapeutic agents and ethanol).
-
Antibiotics to treat infections of the body.
-
Some autoimmune disorders (such as immune thrombocytopenia ).
D) Platelet Dysfunction- This may arise from an intrinsic platelet defect or from an extrinsic factor that changes the function of normal platelets. Dysfunction can be hereditary or acquired. Hereditary disorders of platelet function are namely Von Willebrand disease, the most common hereditary hemorrhagic disease, and hereditary intrinsic platelet disorder. Acquired disorder of platelet dysfunction is commonly due to diseases (e.g., kidney failure) and sometimes to Aspirin (Aspirin minorly increases bleeding in healthy people but may greatly increase bleeding in older patients and those with platelet dysfunction) and other drugs.
What Are the Specific Types of Platelet Disorder?
Some specific types are:
-
Bernard Soulier Disease- There are some very large platelets in the blood that get counted on machines as large red and white blood cells. The platelets that are present lack protein on their surfaces that helps them stick together. It is an inherited condition and is rare.
-
Glanzmann’s Thrombasthenia- The platelets are present in the body but are dysfunctional.
-
Hermansky Padlock Syndrome- The platelets are present but dysfunctional. There are nine different subtypes, of which some are associated with a particular type of albinism (lack of skin pigment).
-
Jacobsen Syndrome (Paris-Trousseau Syndrome)- Platelets are dysfunctional and oversized and are not able to trigger the clotting process.
-
Lowe Syndrome- Only affects males. Many parts of the body are affected, including the eyes, brain, and kidneys.
-
Platelet Release and Storage Pool Defects- Granules present in the platelets help in clotting; when these granules are not stored or utilized effectively, defects happen.
-
Thrombocytopenia With Absent Radius (Tar) Syndrome- Leads to shorter than usual and bent arms as radius bone in both lower arms is absent.
-
Thrombotic Thrombocytopenic Purpura (TTP)- It is a rare condition where small blood vessels are affected due to frequent or abnormal clotting. There is abnormal clotting which results in a low number of platelets in circulation as are taken up by these blood clots.
What Are the Symptoms of Platelet Disorders?
Symptoms of platelet disorder include:
-
Few or more tiny red dot marks (petechiae) appear on the skin of the lower leg region, and small injuries (including needlesticks) appear as black-blue bruises (ecchymoses or purpura).
-
The blood may appear in the nose, stool, or urine.
-
The menstrual cycle or nose bleeding may be heavier than usual.
-
Excessive bleeding after surgery.
What Causes Platelet Disorders?
Some causes for platelet disorders are discussed below:
-
Mutation: Mainly, platelet disorders are associated with a genetic fault or mutation. We have around 30,000 to 40,000 different genes, and they all work together to form new DNA cells. The genes are in pairs, one pair from each parent, on 23 chromosomes- some of these genes get mutated or have faults in them.
-
Inherited: Some of these are autosomal recessive inheritance (faulty genes from both parents), autosomal dominant inheritance (faulty genes from one parent), and x-linked inheritance.
-
Common Drug-Induced: Platelet disorders are most commonly caused by drugs. Drugs, diseases, herbs, and supplements have effects on platelet function.
-
Non-steroidal Anti-inflammatory Medications (NSAIDs): Aspirin, along with other pain-relieving NSAIDs, may prevent platelets from forming clots.
-
Antiplatelet Drugs: Antiplatelet drugs to prevent heart attack and stroke may also prevent clotting and lead to excessive bleeding [Clopidogrel (Plavix), Prasugrel (Effient), and others].
-
Antibiotics: Drugs that treat a bacterial infection, particularly Penicillin, can affect platelet function and cause excessive bleeding.
-
Selective Serotonin Reuptake Inhibitors (SSRIs): Antidepressant medications may prevent blood clotting and cause bleeding, especially when combined with any blood-thinning drugs.
-
Foods, Herbs, Alcohol: Ginkgo biloba, garlic, ginger, turmeric, and several other herbs are known to affect platelet activity and prevent blood clots. Heavy alcohol uptake may also induce thrombocytopenia.
-
Kidney and Liver Disease: Thrombocytopenia is mostly found in people with kidney or liver diseases; people with either disease have a greater risk of excessive bleeding.
-
Lupus: People with lupus (autoimmune disorder) obtain certain antibodies that demolish platelets, resulting in thrombocytopenia.
How Are Platelet Disorders Diagnosed?
-
Any health care personnel will start by taking a proper history of all the previous illnesses encountered by you. They will also ask about the treatments you have undergone and/or medications you are taking.
-
Complete blood count tests are recommended for diagnosing many platelet disorders.
-
A clotting test will be carried out to record the time taken to form a clot.
-
Sometimes, a bone marrow aspiration is also recommended.
-
A low platelet count or platelet dysfunction can be checked by examining the skin for bleeding.
How Are Platelet Disorders Treated?
-
Desmopressin (also known as DDAVP), which works by increasing levels of platelets in the blood by releasing them from storage, is the drug of choice in many cases.
-
Tranexamic acid is given to temporarily boost the proteins that maintain normal blood clots. This is given orally, intravenously, or topically.
-
Important to note that people with a platelet disorder should not use non-steroidal anti-inflammatory drugs (NSAIDs such as Ibuprofen) as this immensely increases the risk of bleeding because NSAIDs reduce platelet sticking together. Other medications for pain relief should be administered.
-
Treatment for some platelet disorders is a stem cell or bone marrow transplant; (only offered in critical conditions.)
-
Intravenous immunoglobulin is commonly administered in immune-related platelet disorders, such as immune thrombocytopenia and neonatal alloimmune thrombocytopenia.
What Is the Prognosis?
The prognosis for people with a platelet disorder depends on the type of disorder they have and its severity. Life-threatening symptoms are treated with medicines. But some undetected platelet disorders show up during specific circumstances, such as surgery, pregnancy, and childbirth which are hard to manage.
In mild to moderate platelet disorders, symptoms are not that prominent, although it is of utmost importance to have knowledge of your platelet disorder, especially around dental surgery. Good dental hygiene is crucial for everyone; it reduces the risk of bleeding gums.
Most people with platelet disorders have healthy lives by staying updated on their treatments and taking everyday precautions to prevent bleeding. Just be sure to talk to your health care provider before beginning a new diet or exercise routine to make sure it is safe for you.
Conclusion
Most known platelet disorders are diagnosed during routine checkups. People generally do not pay any attention until their injury heals slowly or develops large bruises for seemingly no reason. Doctors can diagnose platelet disorders by a simple CBC test report that shows how many platelets you have, what your platelets look like, and how well they clump together. The right treatment for your condition can reduce the risk of future severe bleeding, hazardous blood clots, and long-term health issues.
