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Glanzmann disease

Glanzmann's diasease; Thrombasthenia - Glanzmann

 

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

Causes

 

Glanzmann disease is caused by the lack of a protein that is normally on the surface of platelets. This substance is needed for platelets to clump together to form blood clots.

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

 

Symptoms

 

Symptoms may include any of the following:

  • Heavy bleeding during and after surgery
  • Bleeding gums
  • Bruising easily
  • Heavy menstrual bleeding
  • Nosebleeds that do not stop easily
  • Prolonged bleeding with minor injuries

 

Exams 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. Family members may also need to be tested.

 

Treatment

 

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

 

Outlook (Prognosis)

 

Glanzmann disease is a lifelong condition, and there is no cure. You should take special steps to try 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 by preventing platelets from clumping.

 

Possible Complications

 

Complications may include:

  • Severe bleeding
  • Iron deficiency anemia in menstruating women due to abnormally heavy bleeding

 

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

 

Glanzmann disease is an inherited condition. There is no known prevention.

 

 

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, Anastasi JI, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 152.

Nurden AT, Pillois X, Wilcox DA. Glanzmann thrombasthenia: state of the art and future directions. Semin Thromb Hemost. 2013;39;642-55. PMID: 23929305 www.ncbi.nlm.nih.gov/pubmed/23929305.

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                Review Date: 1/27/2015

                Reviewed By: Yi-Bin Chen, MD, leukemia/bone marrow transplant program, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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