Bleeding disorders: Glanzmann's thrombasthenia
What is Glanzmann's thrombasthenia?
Blood has three main parts: red blood cells, white blood cells and platelets. Red blood cells carry oxygen to your body. White blood cells fight infection. Platelets are small cells that work together to form a clot to stop bleeding. In Glanzmann thrombasthenia, platelets in the blood are missing a protein that helps them stick together. This protein is called Glycoprotein IIb/IIIa. Because of this, the platelets cannot stick together to make a plug to stop bleeding.
What causes Glanzmann's thrombasthenia?
People are born with it. It is inherited in an autosomal recessive pattern. It is very rare and is estimated to be 1 in 1,000,000 births. In Glanzmann's usually both parents are carriers. If both parents are carriers, the person has a 25% chance of having a child with Glanzmann's and a 50% chance that the child will be a carrier. Carriers do not show signs of Glanzmann's. Both boys and girls can have Glanzmann's thrombasthenia.
What are signs of Glanzmann's thrombasthenia?
Symptoms are different for each child. Nose bleeding, bruising, mouth bleeding, heavy periods, heavy bleeding with delivery or surgery is common. Sometimes bleeding is so severe that it is life threatening and the child needs to be hospitalized. The signs and symptoms can change over time. For example, frequent nosebleeds for a young child may decrease as the child reaches adolescence due to the skin in the nose becoming thinker. However, in adolescence, females may have heavy periods also called menorrhagia.
How is it diagnosed?
It is usually diagnosed before the age of five years. The diagnosis is made from the symptoms such as nosebleeds and bruising, as well as Platelet Aggregation studies. Platelet Aggregation studies look at how the platelets work.
What is the treatment?
There are many possible treatments. Your medical team will help choose what works best to stop bleeding. Treatment may include:
- Local control: Direct pressure, nasal tampons, topical thrombin or Nosebleed QR (quick relief powder) may be used.
- Medications: Amicar, Tranexamic Acid, oral birth control pills are sometimes used. Occasionally factor VIIa, a special type of factor concentrate, may be used to prevent or stop bleeding.
- Blood products: Platelets are sometimes given.
Can you prevent bleeding?
There are ways to prevent bleeding. For bruising, some families sew cushioning into pant legs at the knees or shins. For nose bleeds you can use room humidifiers, lubricant or estrogen cream to the nose or normal saline nasal spray. When it is freezing outside, wearing a neck warmer over the nose so the child can breathe moist air may also help. Sometimes factor concentrates such as factor VIIa or Humate P is prescribed on a regular basis to prevent bleeding.
How should I care for my child?
- Visit the hematology clinic at least twice per year and once a month in winter due to frequent nosebleeds.
- Maintain good dental care.
- Use helmets for biking and other sports that require a helmet, and avoid intensive contact sports.
- Avoid aspirin or nonsteroidal anti-inflammatories (NSAIDs) such as ibuprofen or any medicines that contain these products.
- Watch for medicines that affect how platelets work. This includes traditional medicines and some herbal remedies. Contact your nurse or hematologist for a list of medicines to avoid.
- When should I call the clinic?
Call the clinic if you have concerns. Please call if you see any of the any of the following:
- Your child has prolonged bleeding such as nose bleeding despite measures you have tried at home (talk to your health care team to get an idea of how long you should wait before calling).
- Your child is in a car accident or has major trauma.
- Your child has swelling/redness of the muscle or skin, excessive bruising or petechia (small purple pinpoint dots on the skin)
- Your child is going to have any invasive procedure such as surgery, dental work, or stitches.
- A potential carrier for Glanzmann's is pregnant so we can advise the ob/gyn provider on special procedures.
- Your child hits his or her head hard on a hard surface. Usually internal head bleeding does not occur with platelet disorders, but it is wise to watch for change in condition if this occurs.
- If your child has an implantable venous access device (such as a Port a Cath, Hickman) and experiences any issues with it
- Your child has a fever higher than 101° F.
Children's Hematology Clinic 612-813-5940:
- During the weekday, ask to speak with the hematology nurse.
- During nights and weekends, call the hematologist on call.
Call 911 if:
- Your child has a serious injury.
- Your child has any prolonged bleeding that results in not being able to arouse your child.
- Your child is hit in the head and you see a change in behavior such as drowsiness, vomiting, dizziness, blurred or double vision.
This is not specific to your child, but provides general information. If you have any questions, please call your clinic.
Children's Hospitals and Clinics of Minnesota
2525 Chicago Avenue South
Minneapolis, MN 55404
Last Reviewed 7/2015 © Copyright
This page is not specific to your child, but provides general information on the topic above. If you have any questions, please call your clinic. For more reading material about this and other health topics, please call or visit Children's Family Resource Center library, or visit www.childrensmn.org/educationmaterials.
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