DISCLOSURE: information has been provided through the hematology department at the University of Alberta and Edmonton Kaye Clinic. Supplemental information is from the Mayo Clinic, webMD and additional online sources.
Everyone wants to know the facts – the thing that solidifies something within our brains. We want to plant all of the information into our knowledge gardens and allow the roots to grow ever so thick.
But the hardest part about getting the correct information is knowing that the underlying nature of the illness is indefinite. You are always given more what ifs, maybes, differences, etc. than you are given a straight answer.
Because of that, here is EVERYTHING that I have compiled about the notorious purple invader known as ITP.
At the time when this illness was found, platelets were unknown to physicians in 1735. The name Werlhof’s disease was used to describe it at the time. It was only in 1951 that the name was changed to Idiopathic Thrombocytopenic Purpura (also known as Immune Thrombocytopenic Purpura or Immune Thrombocytopenic).
Splenectomy is the front of the line treatment. Corticosteriods were introduced as treatment and later in the 1960s a number of other immune suppressing agents were used. IvIg (Intravenous Immunoglobulin) began in the 80s. Rituximab, known to put many suffers into remission, only became a treatment in the 2000s.
ITP may have NO signs or symptoms, however, there are a few to look for:
- easy or excessive bruising (purpura)
- superficial bleeding into the skin that appears as a small pinpoint reddish-purple spot (petechiae)
- bleeding from the nose or gums
- blood in the urine or stool
- black mouth blisters
So, why does it happen?
Every case is different.
Sometimes it is caused by the immune system mistakenly attacking and destroying platelets (this is why the removal of the spleen is key). If this does not help the illness go away, the I goes from immune to idiopathic meaning “of unknown causes.”
Platelets, what are they really?
Thrombocytes are made in your bone marrow along with your white and red blood cells. Under the microscope, platelets look like a tiny plate – hence the name platelets. Once they are made and circulated into your bloodstream, they live for up to ten days.
These tiny blood cells help your body form clots to stop bleeding. If one of your vessels gets damaged, it sends out multiple signals that are picked up by your platelets and allows them to rush to the scene of the crime to form a plug or clot.
A normal platelet count is between 150 and 450. People with ITP have counts below 20. And since the platelets help the blood clot, as their number gets significantly lower, your risk of bleeding increases. The greatest risk is when your counts fall below 10 – at this point, internal bleeding may occur even without an injury.
Steps to Diagnosis
Diagnosis can ONLY be done by blood work that is ordered by your physician. If multiple abnormalities arise, this is when a specialist is contacted to get more insight. This insight then leads to multiple other tests that include, but are not limited to, blood work, CT scan, MRI, bone marrow aspiration, specialized blood work, genetic testing, etc. The diagnosis is then given by a process of elimination by getting rid of all the other possible causes for why the platelets may be low. This is because a number of other potential conditions have the exact same representation in your blood work (Hep C, HIV, Heliobacter Pylori, Quinine, Anemia, Leukemia, Lymphoma, Castleman’s Disease, etc).
ITP can develop at any age, in any ethnic or racial group, in either gender. It is slightly more common in women but that doesn’t really mean much at the end of the day. ITP can develop suddenly in children following vaccinations or illnesses. In adults, ITP is likely to clear up without any treatment and when it doesn’t, it usually becomes chronic. ITP has NO cure although there are numerous treatments.
There is no single cause but there are multiple triggers:
- colds and flus
You said treatment, tell me MORE!
Most commonly used now is TPO drugs which have been specifically developed to target ITP. These have been available since 2008 and each individual case has a different result, but mostly have a great impact. The TPO drugs are Revolade (Eletrombopag) and N-Plate.
These drugs are designed to stimulate platelet production and therefore attack the problem of ITP in a new way than the traditional treatments like cortiocosteroids, azathioprine, rituximbad, cyclosporine, etc., which are just immune suppressing medication.
Other treatments include IvIg and Anti-D blockers. Splenectomy, which is now used very rarely unless the spleen is enlarged or other options have failed.
Nature vs. Nurture
Something that has been found is that anywhere from 30-50% of the cause of ITP is genetic, but that doesn’t necessarily mean that if you have the genetic markers you will develop ITP.
Meaning you HAVE to have the genetic makeup in the first place to develop ITP BUT you also have come into contact with some environmental trigger in order for it to develop.
These factors include:
- environmental – stress, lifestyle
- other autoimmune disorders or illnesses
- Heliobacter Pylori bacteria
The difficulty is that for each individual sufferer, it is almost impossible to detect which one or combination of the above factors ultimately triggered ITP.
Since there is no one size fits all cure, the ongoing research is focusing on recording responses to treatments to get better at predicting which treatment is most likely to work best for each individual.
It’s safe to say that there is still a long way to go.
The more knowledge that is acquired, the more confirmation one gets that ITP is much more complex than it was previously thought.