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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

Glanzmann's disease is caused by the lack of a protein that is normally on the surface of platelets. This substanceis needed for the cells 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

Exams and Tests

The following tests may be used to diagnose this condition:

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.

Outlook (Prognosis)

Glanzmann's thrombasthenia is a life-long condition for which there is no cure. You should take special steps to avoid bleeding if you have this condition.

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.

Possible Complications

When to Contact a Medical Professional

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 platelet defect. You may wish to seek genetic counseling if you have a family history of this problem and are considering having children.

References

Macartney CA, Paredes N, Chan AKC. Disorders of Coagulation in the Neonate. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Saunders Elsevier; 2012:chap 152.

Nichols WL.Von Willebrand Disease and Hemorrhagic Abnormalities of Platelet and Vascular Function. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 176.


Review Date: 3/3/2013
Reviewed By: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.
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