A Member of the University of Maryland Medical System   |   In Partnership with the University of Maryland School of Medicine

Share

Email PageEmail Print PagePrint

Home > Medical Reference > Encyclopedia (English)

Toggle: English / Spanish

Congenital platelet function defects - Overview

Alternative Names

Platelet storage pool disorder; Glanzmann's thrombasthenia; Bernard-Soulier syndrome; Platelet function defects - congenital

Definition of Congenital platelet function defects:

Congenital platelet function defects are problems with platelets, one of the blood elements needed for normal blood clotting. Congenital means present from birth.

Causes, incidence, and risk factors:

Platelets are elements in the blood that help form clots. Congenital platelet function defects are bleeding disorders that cause reduced platelet function, even though there are normal platelet counts.

People with these disorders usually have a family history of a bleeding disorder that causes prolonged bleeding after minor cuts or surgery, or easy bruising.

  • Bernard-Soulier syndrome occurs when platelets lack a substance that sticks to the walls of blood vessels. This disorder may cause severe bleeding.
  • Glanzmann's thrombasthenia is a condition caused by the lack of a protein needed for platelets to clump together. This disorder may also cause severe bleeding.
  • Platelet storage pool disorder (also called platelet secretion disorder) is due to one of several defects that cause easy bleeding or bruising. It is caused by the faulty storage of substances inside platelets. These substances are usually released to help platelets function properly.
  • Reviewed last on: 2/28/2011
  • David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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:chap141.

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.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com
 
Adam QualityA.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com
Connect with UMMC
Facebook Twitter YouTube Blog iPhone

Please rate the quality of this article.

Do you find this article to be helpful / informative?
              
Poor                                       Excellent

Do you have any brief comments on this page: (up to 255 characters)

© 2011 University of Maryland Medical Center (UMMC). All rights reserved.
UMMC is a member of the University of Maryland Medical System,
22 S. Greene Street, Baltimore, MD 21201. TDD: 1-800-735-2258 or 1.866.408.6885