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Glanzmann’s disease, also called Glanzmann’s thrombasthenia, is a rare condition in which your blood doesn’t clot properly. It’s a congenital hemorrhagic disorder, meaning that it’s a bleeding disorder present at birth.
Glanzmann’s disease results from not having enough glycoprotein IIb/IIIa (GPIIb/IIIa), a protein usually found on the surface of blood platelets. Platelets are small blood cells that are the first responders in the case of a cut or other bleeding injury. They normally clump together to form a plug in the wound and stop bleeding.
Without enough glycoprotein IIb/IIIa, your platelets won’t be able to stick together, or clot, properly. People who have Glanzmann’s disease have difficulty getting their blood to clot. Glanzmann’s disease can be a serious issue during surgeries or in the case of major injuries because a person could lose large quantities of blood.
The genes for glycoprotein IIb/IIIa are carried on chromosome 17 of your DNA. When there are defects in these genes, it can lead to Glanzmann’s.
This condition is autosomal recessive. That means both of your parents must carry the defective gene or genes for Glanzmann’s in order for you to inherit the disease. If you have a family history of Glanzmann’s disease or related disorders, you have an increased risk of inheriting the disorder or passing it on to your children.
Doctors and scientists are still researching what exactly causes Glanzmann’s disease and how it can best be treated.
Glanzmann’s disease may cause severe or continual bleeding, even from a minor injury. People who have the disease may also experience:
Your doctor may use the following simple blood tests to help diagnose Glanzmann’s disease:
Your doctor may also test some of your close relatives to check if they have Glanzmann’s disease or any of the genes that may contribute to the disorder.
There are no specific treatments for Glanzmann’s disease. Doctors may suggest blood transfusions, or injections of donor blood, for patients who have severe bleeding episodes. By replacing damaged platelets with normal platelets, people with Glanzmann’s disease often have less bleeding and bruising.
You should avoid medications such as ibuprofen, aspirin, blood thinners such as warfarin, and anti-inflammatory drugs. These drugs are known to prevent platelets from clotting and can cause further bleeding.
If you’re receiving treatment for Glanzmann’s disease and notice that your bleeding is not stopping or is worsening, you should talk with your doctor.
Glanzmann’s disease is a long-term disorder with no cure. There are many dangers of continual bleeding such as chronic anemia, neurological or psychiatric problems, and possibly death, if enough blood is lost. People with Glanzmann’s have to be very careful when they are injured and bleeding occurs. Women who have the condition may develop iron deficiency anemia during their menstrual cycles.
If you begin to bruise easily or bleed for unknown reasons, you should talk with your doctor. It may mean the disease is becoming worse or that there’s another underlying condition that your doctor needs to diagnose.
A blood test can help detect the genes responsible for causing Glanzmann’s disease. If you have a family history of any platelet disorders, it may be helpful to seek genetic counseling if you’re planning to have children. Genetic counseling can help you determine the potential risk of your child inheriting Glanzmann’s disease.
Written by: Elly Dock
Published on: May 30, 2012
Medically reviewed on: Feb 17, 2016: Mark Laflamme, MD
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