Definition

What is Glanzmann disease?

Glanzmann disease, also called Glanzmann 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 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 disease have difficulty getting their blood to clot. Glanzmann disease can be a serious issue during surgeries or in the case of major injuries because a person could lose large quantities of blood.

How common is Glanzmann disease?

Glanzmann disease is a rare disorder that affects males and females in equal numbers. Approximately 300 cases have been reported. This condition occurs with greater frequency in populations in which intermarriage within a group (consanguinity) is more prevalent such as in some regions of the Middle East, India, and France. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Glanzmann disease?

Glanzmann disease may cause severe or continual bleeding, even from a minor injury. People who have the disease may also experience:

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you or your loved one has any signs or symptoms listed above or you have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Glanzmann disease?

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 disease.

Risk factors

What increases my risk for Glanzmann disease?

This condition is autosomal recessive. That means both of your parents must carry the defective gene or genes for Glanzmann disease in order for you to inherit the disease. If you have a family history of Glanzmann disease or related disorders, you have an increased risk of inheriting the disorder or passing it on to your children.

Please consult with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Glanzmann disease diagnosed?

Doctor may use the following simple blood tests to help diagnose Glanzmann disease:

  • Platelet aggregation tests: To see how well your platelets clot
  • Complete blood count: To determine the number of blood platelets you have
  • Prothrombin time: To determine how long it takes for your blood to clot
  • Partial thromboplastin time: Another test to see how long it takes for your blood to clot

Doctor may also test some of your close relatives to check if they have Glanzmann disease or any of the genes that may contribute to the disorder.

How is Glanzmann disease treated?

There are no specific treatments for Glanzmann 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 disease often have less bleeding and bruising.

If you’re receiving treatment for Glanzmann disease and notice that your bleeding is not stopping or is worsening, you should talk with your doctor.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Glanzmann disease?

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 have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: February 5, 2018 | Last Modified: February 5, 2018

Want to live your best life?
Get the Hello Doktor Daily newsletter for health tips, wellness updates and more.