Disease Information for Glanzmann's thrombasthenia

A congenital bleeding disorder with prolonged bleeding time, absence of aggregation of platelets in response to most agents, especially ADP, and impaired or absent clot retraction; Platelet membranes are deficient in or have a defect in the glycoprotein IIb-IIIa complex (PLATELET GLYCOPROTEIN GPIIB-IIIA COMPLEX)----------------------------------Glanzmann Thrombasthenia; Diacyclothrombopathia IIb-IIIa; GP IIb-IIIa Complex, Deficiency of; GTA;

Glanzmann Disease; Glanzmann Thrombasthenia; Glanzmann Thrombasthenia, Type A; Glanzmann-Naegeli Syndrome;

Glycoprotein Complex IIb/IIIa, Deficiency of; Platelet Fibrinogen Receptor Deficiency; Thrombasthenia;

Thrombasthenia of Glanzmann and Naegeli;

Glanzmann thrombasthenia (GT) is a rare inherited blood clotting (coagulation) disorder characterized by the impaired function of specialized red blood cells (platelets) that are essential for proper blood clotting; Symptoms of this disorder may include abnormal bleeding and/or hemorrhage; The symptoms are not progressive, however, prolonged untreated or unsuccessfully treated hemorrhaging associated with Glanzmann thrombasthenia may be life threatening [NORD 2005]--------------------------------------------Glanzmann"s thrombastenia

Nägeli syndrome II; Révol"s syndrome; Athrombocytopenic purpura; congenital hemorrhagic thrombocytic dystrophy; diacyclothrombopathy, glycoprotein IIb (GP IIb-III) complex deficiency; hemorrhagic thrombasthenia; hereditary thrombasthenia; hereditary thrombocytopenic purpura; platelet fibrinogen receptor deficiency; platelet glycoprotein IIb-III deficiency; thrombasthenia; thrombocytasthenia; thrombocytopathic purpura;

A rare congenital abnormality of blood platelets, characterized by easy bruising and excessive bleeding after trauma and epistaxis; The platelet membrane lacks specific glycoproteins Iib and IIIa which are the receptor of fibrinogen; Bleeding is prologed, clot retraction is diminished, and platelets do not aggregate during blood coagulation or after addition of ADP (adenosine diphosphate); Inheritance is mostly autosomal recessive, but some cases are known to be transmitted as an autosomal dominant trait; An acquired form is seen particularly in malignant systemic diseases and uraemia; Both sexes affected; onset from birth; May be asymptomatic until trauma or surgical procedures make condition evident;--------------------[whonamedit_com 2005]---------

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