Glanzmann's disease

Definition

Glanzmann’s disease is a rare disorder of blood platelets, which results in easy bruising and nosebleeds.

Alternative Names

Thrombasthenia

Causes, incidence, and risk factors

Glanzmann’s disease is caused by lack of a protein required for platelets to clump together (aggregate) normally.

The condition is congenital, which means it is present from birth.

Symptoms

• Abnormal menstrual periods
• Bleeding during and after surgery
• Bleeding gums
• Easy bruising
• Nosebleeds (epistaxis)
• Prolonged bleeding with small injuries

Signs and tests

The following tests may be used to diagnose this condition:

• Complete blood count (CBC)
• Bleeding time
• Platelet aggregation tests
• Prothrombin time (PT) and partial thromboplastin time (PTT)

Other tests may be necessary, including the testing of relatives.

Treatment

There is no specific treatment for this disorder. Platelet transfusions may be given to patients who are having severe bleeding.

Expectations (prognosis)

Glanzmann’s thrombasthenia is a lifelong condition for which there is no cure. Patients should take precautions to avoid bleeding.

Anyone with a bleeding disorder should avoid taking aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen because these drugs can prolong bleeding times.

Complications

• Severe bleeding
• Iron deficiency anemia in menstruating women

Calling your health care provider

Call your health care provider if bleeding or bruising of an unknown cause is present, or if bleeding does not stop after usual treatments.

Prevention

A blood test can detect the gene responsible for the condition.

Genetic counseling may be helpful to couples with a family history of platelet disorders who are planning to have children in the future.

References

McMillan R. Hemorrhagic disorders: Abnormalities of platelet and vascular function. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 179.