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Thrombasthenia (Glanzmann 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).
Also Known As:
Glanzmann Thrombasthenia; Deficiency of GP 2b 3a Complex; GP IIb-IIIa Complex, Deficiency Of; Glanzmann Thrombasthenia, Type A; Glycoprotein Complex IIb-IIIa, Deficiency Of; Platelet Fibrinogen Receptor, Deficiency of; Platelet Glycoprotein 2b-3a Deficiency; Platelet Glycoprotein IIb-IIIa Deficiency; Thrombasthenia of Glanzmann and Naegeli; GP IIb IIIa Complex, Deficiency Of; Glycoprotein Complex IIb IIIa, Deficiency Of; Platelet Glycoprotein 2b 3a Deficiency; Platelet Glycoprotein IIb IIIa Deficiency; Thrombasthenia, Glanzmann; Thrombasthenias
Networked: 851 relevant articles (20 outcomes, 56 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Hemorrhage
2. Bernard-Soulier Syndrome (Giant Platelet Syndrome)
3. von Willebrand Diseases (von Willebrand's Disease)
4. Hemophilia A (Haemophilia)
5. Angiodysplasia

Experts

1. Nurden, Alan T: 18 articles (12/2019 - 03/2002)
2. Poon, Man-Chiu: 11 articles (03/2021 - 01/2006)
3. Nurden, Paquita: 11 articles (12/2019 - 03/2002)
4. Rosenberg, Nurit: 10 articles (01/2019 - 06/2003)
5. Nurden, A T: 10 articles (06/2013 - 10/2001)
6. Nurden, P: 10 articles (06/2013 - 05/2000)
7. Pillois, Xavier: 9 articles (12/2019 - 09/2011)
8. Di Minno, Giovanni: 9 articles (07/2019 - 12/2004)
9. Seligsohn, Uri: 9 articles (01/2019 - 09/2002)
10. Fiore, Mathieu: 8 articles (01/2022 - 09/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Thrombasthenia:
1. recombinant FVIIa (rFVIIa)FDA Link
2. AntibodiesIBA
3. Factor VIIa (Activated Factor VII)IBA
4. IntegrinsIBA
5. Glycoproteins (Glycoprotein)IBA
6. Tranexamic Acid (AMCA)FDA Link
7. Platelet Glycoprotein GPIIb-IIIa ComplexIBA
8. ThrombinFDA Link
9. IsoantibodiesIBA
10. LigandsIBA
06/04/2004 - "In addition, it suggests mechanistic hypotheses pertaining to both naturally occurring mutations responsible for Glanzmann thrombasthenia and to point mutations that affect ligand binding."
11/14/1997 - "Disruption of the ligand binding domain by the Glanzmann thrombasthenia mutation beta3-D119Y constitutively abolished migration toward both Vn and Fn, and attachment to Vn but not to Fn. "
05/01/2001 - "Of the six, only the S(752)P substitution, which also characterizes a form of the human bleeding disorder Glanzmann's thrombasthenia, fails to rescue beta 3(-/-) osteoclasts or restore ligand-activated signaling in the form of c-src activation. "
10/01/2004 - "The method provides a quantitative assessment of the physical and antigenic properties of platelets (e.g., surface expression of receptors, bound ligands, components of granules, or interactions of platelets with other platelets, other blood cells, or components of the plasma coagulation system), thereby facilitating the diagnosis of inherited or acquired platelet disorders (e.g., Bernard-Soulier syndrome, Glanzmann thrombasthenia, storage pool disease), the pathological activation of platelets (e.g., in the setting of acute coronary syndromes, cerebrovascular ischemia, peripheral vascular disease, cardiopulmonary bypass), and changes in the ability of platelets to activate via specific stimuli (e.g., efficacy of antiplatelet therapies). "
06/01/2013 - "As in our previous report of an R560β(3) mutation in a patient with Glanzmann thrombasthenia, R560β(3) murine platelets spontaneously bound antibody that only recognizes activated αII b β3 bound to its ligand, fibrinogen. "

Therapies and Procedures

1. Platelet Transfusion (Blood Platelet Transfusions)
2. Therapeutics
3. Hematopoietic Stem Cell Transplantation
4. Cardiopulmonary Bypass (Heart-Lung Bypass)
5. Tonsillectomy