Essential thrombocythemia: Too many platelets can be a problem!

Platelets are typically considered as the small blood cells that help the blood clot. It may appear that the more platelets you have the better you will be protected from bleeding.

Unfortunately, the body doesn’t work quite that simply.

Essential thrombocythemia or ET is a rare chronic blood disorder that is characterized by an abnormal increase in the number of platelets produced by the bone marrow. It is a type of myeloproliferative neoplasms.

The interesting thing about ET is that an excess of platelets can lead to increased blood clots.

Patients can experience headaches, dizziness, visual disturbances, burning or redness in hands and feet, or symptoms of thrombosis. Others, however, do not have any symptoms and find out about the condition when their routine blood test reveals a chronic high platelet count.

There’s another strange thing, too.

Though the risk for clots is greater, some patients may also experience bleeding, especially if the platelet count is very high. This may be associated with acquired von Willebrand syndrome, in which the extremely elevated platelet count disrupts the normal clotting ability.

The high platelet count is not sufficient to diagnose. Other common causes of thrombocytosis including infection, inflammation or iron deficiency should be considered and ruled out. The genetic testing of mutations like JAK2, CALR and MPL may also assist the diagnosis.

Treatment will vary depending on age, previous thrombosis and overall risk. Medications may be needed to decrease the risk of complications, or some patients may only need to be monitored.

ET is a very interesting reminder that even the most mundane of tests, like a platelet count, can tell a much larger story.

Too much of a good thing can be as bad as too little.

Have you ever come across a patient with an unexpectedly high platelet count and wondered what was causing it?

MBH/PS

found the contrast especially interesting ET shows that a high platelet count doesn’t necessarily mean better protection from bleeding. In fact, excessive platelets can increase the risk of thrombosis, while extremely high counts can paradoxically be associated with bleeding through acquired von Willebrand syndrome.