Immune thrombocytopenia is a disease in which the blood’s platelet cells becomes low because of an immune response against them.
Platelets are the first line of defense against excessive bleeding. Every time your body suffers any kind of trauma, even when it is so mild you don’t even notice it, platelet activation occurs to prevent bleeding.
The medical prefix for anything platelet related is thrombo. The term thrombocytopenic purpura refers to a disease in which a lack of platelets leads to bleeding.
Moreover, immune thrombocytopenic purpura is the most common form of thrombocytopenic purpura. In this disease, your immune system destroys platelets causing a low platelet count.
The disease is more common in children than in adults. Approximately, in the United States, immune thrombocytopenic purpura affects 1 in every 20.000 children and 1 in every 50.000 adults.
Although it sounds like a complicated and intimidating disease, in most cases, it is a mild and self-limited disease that, in many circumstances, doesn’t even require medical treatment. However, it is important to note that severe cases can cause serious, potentially lethal complications like intracranial hemorrhage.
Intracranial hemorrhage is the most frequent cause of death in immune thrombocytopenic purpura patients. It occurs in only 0.5% to 1% of cases; approximately half of those cases are fatal.
Immune thrombocytopenic purpura is more common in boys aged 1 to 6 years. Adults can suffer from the disease at any age, but onset in patients older than 60 is infrequent, those who get it at that age have a higher risk for complications.
I am a medical doctor. In the following pages, I will tell you everything you need to know about this condition.
What is immune thrombocytopenic purpura?
Immune thrombocytopenic is part of a group of diseases called bleeding disorders. In a bleeding disorder, the blood doesn’t clot as it should after trauma, which in turn, causes uncontrollable bleeding.
Blood clotting depends on two separate but equally essential systems, the platelet system and the coagulation cascade. In this case, the problem lies with the platelets.
Platelets are small, colorless cell fragments in the blood. When people bleed, these fragments merge to form a platelet clot that stops the bleeding before the coagulation cascade kicks off to create a permanent clot. In a way, platelets are the first line of defense against bleeding. Platelet production occurs in the bone marrow along with red blood cell and white blood cell production.
In thrombocytopenic purpura, there are low platelet levels, which increase the risk of bleeding. Patients with platelet deficiency often have many purple bruises all over their skin and mucous membranes like the mouth. These purple bruises are the result of blood leaking into the skin. The sum of all these bruises receives the name of purpura.
Therefore, in immune thrombocytopenic purpura, there is no problem with the platelets themselves or with platelet production. As the name says, this disease’s situation is an inappropriate immune response towards platelets that leads to platelet destruction and thrombocytopenia.
What are the two types of Immune thrombocytopenic purpura?
Depending on its duration, ITP can be classified into chronic ITP, persistent ITP, and acute ITP. Acute ITP is the most common presentation in children and lasts for less than three months.
Approximately four out of five children recover completely without treatment in the acute phase between two and eight weeks of disease’s appearance.
Persistent ITP lasts between three and twelve months. In this period, the patients are expected to be cured of the disease. It is essential not to surpass the barrier of the twelve months when it becomes a chronic ITP.
Chronic ITP lasts for more than twelve months and is more common in adults, although children and adolescents can also suffer from it too. This presentation of the disease is chronic because it needs long term monitoring by the doctor. After all, the platelet cells level in the blood does not return to normal levels.
What causes immune thrombocytopenic purpura?
Years ago, immune thrombocytopenic purpura had another name, idiopathic thrombocytopenic purpura. The word idiopathic means that it is of unknown cause.
In the past, researchers didn’t understand well what was the cause of immune thrombocytopenic purpura. Nevertheless, nowadays, the mechanisms that produce the disease are more clear.
In this autoimmune disorder, the immune system produces antibodies that attack platelets. These antibodies mark platelets as targets for destruction.
Importantly, most cases occur a few weeks after an acute viral infection like a cold or the flu in children. The exact mechanisms viruses use to trigger an autoimmune response towards platelets are still under research.
The spleen plays a significant role in thrombotic thrombocytopenic purpura. It is the site of the destruction of antibody-marked platelets. The spleen is full of a group of cells, macrophages, whose primary function is to eat and destroy bacteria, parasites, fungi, and anything marked with the rest antibodies.
Hence, surgical removal of the spleen leads to a normal platelet count in immune thrombocytopenic purpura patients
Does helicobacter pylori initiate or perpetuate immune thrombocytopenic purpura?
Several chronic infections have a link with ITP, including viral infections like hepatitis C and HIV. Studies from several countries, including Italy, Japan, and Korea, have shown that in ITP patients with H. pylori infection, eradicating the bacteria leads to an improvement in platelet count.
However, H. pylori do not appear to be more frequent in ITP patients than in the general population. So although there appears to be some kind of relationship, researchers haven’t established direct causality just yet. Routine testing for H. pylori in all ITP patients is not the standard of care.
What drugs can cause purpura?
Some drugs can cause drug-induced thrombocytopenia, which can be mistaken for immune thrombocytopenic purpura. ITP diagnosis requires the exclusion of drug-related thrombocytopenia.
In drug-related thrombocytopenia, the platelet count returns to normal after discontinuation of treatment. The list of drugs that can cause a low platelet count is huge and includes over 1500 drugs. Here are some of the most common ones:
- Antibiotics: cephalosporins like ceftriaxone
- Gold salts
- Anti-psychotic drugs
- Antiepileptic drugs
Acute and chronic alcohol consumption can also cause thrombocytopenia.
Can stress cause platelets to drop?
Some studies have shown that high levels of stress and anxiety can moderately lower the platelet count. However, stress by itself does not lower platelets to the point of producing bleeding.
What are the symptoms of immune thrombocytopenic purpura?
Depending on the severity of the disease, symptoms can vary greatly. Yet, keep in mind, immune thrombocytopenic purpura is a disease that occurs primarily to an otherwise healthy person. Therefore, signs of wasting, malnutrition, infection, or chronic illness suggest bleeding due to a different cause.
Most signs of this disease can exist on the skin and mucus membranes. Common skin signs include pinpointing size purple spots called petechiae.
Petechiae are more common in the legs, but they can appear at any part of the body. Also, they can appear after putting mild to moderate pressure in a restricted area; for example, petechiae can develop just after blood pressure measuring in the area where the cuff was.
Patients that have only petechiae have a relatively low risk of severe bleeding. Other skin symptoms like widespread bruises (ecchymosis), prolonged bleeding after taking a blood sample, and blood-filled papules indicate a greater bleeding risk.
The most common mucosal symptom is abnormal bleeding from the gums after brushing your teeth.
Other more severe symptoms can also include:
- Prolonged bleeding during cuts
- Prolonged bleeding during surgery
- Spontaneous, unexplainable nosebleeds
- Blood in the urine
- Blood in the stool
- Abnormally heavy menstruation
In some cases, there is immune destruction of platelets in the spleen, although, of too small a magnitude to produce any symptoms; this is asymptomatic immune thrombocytopenic purpura. It is usually an incidental finding in a routine blood test.
What are the signs of internal bleeding?
Internal bleeding is the most feared complication of ITP, and the worst form of internal bleeding is intracranial bleeding. It can exist after mild to moderate head trauma that wouldn’t cause bleeding in a person with a normal platelet level.
Besides, in severe cases, intracranial bleeding can happen spontaneously. Signs of intracranial bleeding include:
- Focal neurological symptoms like in a stroke: One-sided weakness or paralysis, loss of sensibility or tingling in one side of the body, facial paralysis, difficulty talking, incoherent words, loss of consciousness, loss of balance, or altered gait.
- Slow heart rhythm: It is a complication of increased intracranial pressure due to the bleeding.
- High blood pressure: It is also due to increased intracranial pressure.
- Severe headache: Many authors describe headaches secondary to intracranial bleeding as one of the worst physical pain imaginable.
- When a doctor examines a patient’s retina with intracranial pressure with an ophthalmoscope, he might see hemorrhages and blurring of the optic nerve.
- Pupils of different sizes or inability to make normal eye movements.
Can ITP cause leukemia?
No, ITP does not progress into leukemia. What does happens many times is that a person with leukemia is mistakenly diagnosed with ITP. In the early stages, leukemia can manifest itself as thrombocytopenia and no other observable symptoms.
How is immune thrombocytopenic purpura diagnosed?
Immune thrombocytopenic purpura is an exclusion diagnosis, which means that diagnosis requires the presence of persistent low platelet levels in the absence of another medical explanation. Ruling out other conditions requires a good history, physical exam, and extensive testing.
The first step in diagnosing immune thrombocytopenic purpura is doing a complete blood count. The only altered value on that test should be a decreased number of platelets (isolated thrombocytopenia).
Any other finding, such as anemia, too little, or too much white blood cells, should point the physician towards another diagnosis. If both anemia and low platelet count are present, your doctor might consider Evans Syndrome.
In Evans syndrome, there is immune thrombocytopenic purpura plus autoimmune hemolytic anemia; this is an idiopathic condition.
The second step is a peripheral blood smear; in this test, a hematologist observes the blood under the microscope to see if there are any abnormalities in the blood cells’ shape. Typically in ITP, platelets have a normal size and shape.
However, there may be a small percentage of abnormally large platelets. If most of the platelets are abnormal, the diagnosis is more likely to be bone marrow disease than immune thrombocytopenic purpura.
Testing for platelet specific antibodies is only available in some reference medical centers and main laboratories. The reliability of the results strongly depends on the laboratory. The absence of platelet specific antibodies is not enough to exclude the diagnosis of ITP, and testing is not required for diagnosis.
It is important to note that many authors recommend against routinely measuring antiplatelet antibodies in patients with probable ITP.
What is a bone marrow biopsy?
A bone marrow biopsy is a procedure to extract and observe the soft red puffy substance inside your bones, the bone marrow.
The bone marrow is the body’s setting where the production of all the blood cells takes place, including red blood cells, white blood cells, and platelets. A bone marrow biopsy helps show whether your bone marrow is healthy and making a normal amount of blood cells.
Because in ITP, trouble happens after the platelets leave the bone marrow, a biopsy should be normal. However, many cases of ITP require a bone marrow biopsy to rule out other diseases, leukemia, for example.
A bone marrow biopsy is a safe procedure that usually takes around 10 minutes and is generally performed by a hematologist. It involves making a small incision in the bone and then inserting a hollow needle to extract the bone marrow. The patient will be awake with local anesthesia at the site of the incision.
Although in most cases, it is an uncomplicated procedure, it can have complications such as:
- Excessive bleeding on the incision site
- Bone infection
- Long-lasting discomfort on the biopsy site
- Penetration of the breastbone and injury to the heart and lungs
To prevent these complications, most doctors try to avoid biopsy when possible.
So far, the value of bone marrow for ITP diagnosis isn’t fully established. More data is needed to develop clear guidelines. In general, a bone marrow biopsy is not necessary when the complete blood cell count shows isolated thrombocytopenia, the peripheral blood smear shows no abnormalities, and the patient exhibits classic ITP symptoms.
It is not the case for patients with systemic symptoms such as fever or weight loss, abnormalities in the peripheral blood smear, or deficiency of more than one blood cell line ( thrombocytopenia and anemia, for example).
Is immune thrombocytopenic purpura curable?
Unfortunately, immune thrombocytopenic purpura is not a curable condition. Despite this, the outcomes tend to vary.
For example, many patients experience an improvement in their platelet count after treatment. Some patients even go through spontaneous remission without treatment. Other patients report that changing their diet and lifestyle habits can make them feel better.
How is immune thrombocytopenic purpura treated?
The main objective in ITP treatment is to improve the platelet count to a safe level to allow patients to live a normal life without the dear of bleeding to death after mild trauma. However, after platelet-level normalization, relapses may occur.
Currently, systemic corticosteroids are the standard of care for the initial management of acute ITP. Corticosteroids suppress the immune system, preventing the destruction of more platelets. Steroids also improve clotting, preventing further bruising and bleeding. Some of the most commonly used steroids include:
Intravenous immunoglobulin (IVIG) is the drug of the second choice after corticosteroids. Corticosteroids have similar efficacy and a lower rate of side effects than IVIG. Therefore, IVIG is only for cases refractory to corticosteroid treatment or patients with contraindications for corticosteroid use.
Most children with ITP do not require treatment, and the thrombocytopenia resolves spontaneously. The American Society of Hematology does not recommend treatment for children with only minor bleeding.
Cases of mild ITP can be left under observation alone. In adults, guidelines recommend treatment only for patients with a platelet level below 30.000 (the normal range is 150.000 to 400.000).
Short corticosteroid courses (of less than six weeks) are preferred over long term courses to avoid complications like immunosuppression, hypertension, and Cushing syndrome.
In cases were both corticosteroids and IVIG prove ineffective after months of treatment, the next step is to remove the spleen (splenectomy). Splenectomy usually results in rapid, complete, and long-lasting remission. However, relapses after five years are not uncommon.
The splenectomy will result in a lifelong increased risk of sepsis from infection by encapsulated bacteria. For this reason, the procedure is reserved only for selected patients, resistant to treatment, and with a high risk of severe bleeding.
What is the prognosis for an ITP patient?
Over 80% of children and 60% of adults have a spontaneous recovery during the first 2 to 8 weeks. Fatal bleeding occurs in only 0.9% of cases. However, some cases progress to chronic ITP.
Risk factor for chronicity include:
- Female gender
- No preceding infection or vaccination
- A platelet count below 20.000 at presentation
- Requires treatment with bot methylprednisolone and IVIG at the onset
- Presence of antinuclear antibodies (autoantibodies directed towards the cell nuclei, which are heavily associated with autoimmune disease).
Are you or your child having symptoms of it?
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