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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 the lack of a protein that is normally on the surface of platelets, which is needed for them to clump together (aggregate) normally.

The condition is congenital, which means it is present from birth. There are several genetic abnormalities that can cause the condition.

Symptoms

Bleeding during and after surgery
Bleeding gums
Easy bruising
Heavy menstrual bleeding
Nosebleeds (epistaxis)
Prolonged bleeding with minor 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 needed, 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 life-long 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. These drugs can prolong bleeding times and prevent platelets from activating and aggregating.

Complications

Severe bleeding
Iron deficiency anemia in menstruating women

Calling your health care provider

Call your health care provider if:

You have bleeding or bruising of an unknown cause
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

Bennett JS. Hereditary disorders of platelet function. In: Hoffman R, Benz EJ Jr., Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier; 2008:chap 141.

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.

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