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Glanzmanns Disease

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Intro/History Glanzmanns disease also known as, Thombasthenia-Glanzmann is a rare disorder of blood platelets, which result in easy bruising and nosebleeds, and was first described in 1918. Glanzmanns disease is a congenital hemorrhagic disorder, meaning it's a bleeding disorder present at birth. Glanzmanns disease results from not having enough glycoprotein, a protein usually found on the surface of blood platelets. Platelets are small blood cells that are the first responders in the case of a cut or injury, they normally clump together to form a plug in the wound and stopped bleeding. Glanzmanns Disease
Glanzmanns disease is a hereditary disease. Glanzmanns disease has differing severity. In type I there is a severe less than 5% deficiency …show more content…

Platelet transfusions may be given to people who are having severe bleeding episode. Minor bleeding from sites that can be seen, such as teeth and gums can be controlled by a local application of thrombin. Nosebleeds may require nasal packing (cocaine has been used on packing material) as well as platelet transfusion. Antifibrinolytic drugs (rare) may help control bleeding and tooth decay. Heavy menstrual cycles may require hormonal therapy to help control bleeding. Patients are likely to require multiple transfusions during their lifetime; doctors try to avoid platelet alloimmunization. Prevention of alloimmunization is best accomplished by using leukocyte depleted blood products. The only way to accomplish blood products with leukocyte depletion is with mechanical filtration. Only filtered blood products should be given. Another attempt in preventing alloimmunization is the use of HLA- matched platelets. Patients should be vaccinated for hepatitis B, due to the risk of infection associated with multiple transfusions. Patients should always avoid medications that affect platelet functions, such as aspirin in known blood thinners like warfarin. They should also avoid any nonsteroidal anti-inflammatory drugs (NSAID) such as ibuprofen and naproxen. These drugs can prolong bleeding …show more content…

The defects seen in Glanzmann disease are split equally between glycoprotein IIb and glycoprotein IIIa.

References

McCartney 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 www.nlm.nih.gov/medlineplus/ency/article www.nlm.nih.gov/medlineplus/encylopedia.html

Nurden AT, Pillois X, Wilcox, DA. Glanzmanns thrombasthenia: state of the art, and future directions. Semin Thromb Hemost. 2013; 39: 642-55 www.nci.nlm.nih.gov/pubmed 3/1/16 12:43pm
Updated January 27, 2015
Updated by: Yi-Bin Chen, MD
Leukemia/ bone marrow Transplant program, Massachusetts General Hospital, Boston, MA.
Reviewed by David Zieve, MD MHA, ISIA Ogilvie, PhD and the A.D.A.M editorial team.

Glanzmanns thrombasthenia. (2016, Jan. 18). Retrieved from www.ghr.nlm.nih.gov/condition/glanzmann-thrombasthenia.

Nurden, AT (2006). Glanzmanns thrombasthenia. Orphanet journal of rare diseases, 1 (10). Retrieved from

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