Thrombasthenia (Glanzmann Thrombasthenia)

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559 relevant articles (12 outcomes, 38 trials/studies) found for this Disease

Description: 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; Thrombasthenia, Glanzmann; Thrombasthenias

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases

Hematologic Diseases: 1480

Hemorrhagic Disorders: 456

Thrombasthenia: 559

Blood Coagulation Disorders: 88

Inherited Blood Coagulation Disorders: 34

Thrombasthenia: 559

Blood Platelet Disorders: 78

Thrombasthenia: 559

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 449

Inborn Genetic Diseases: 578

Inherited Blood Coagulation Disorders: 34

Thrombasthenia: 559

Related Diseases

1.	Hemorrhage
2.	Bernard-Soulier Syndrome (Giant Platelet Syndrome)
3.	Neonatal Alloimmune Thrombocytopenia
4.	Acute Coronary Syndrome
5.	Peripheral Vascular Diseases (Peripheral Vascular Disease)

Experts

1.	Rosenberg, Nurit: 5 articles (05/2007 - 06/2003)
2.	Nurden, P: 4 articles (03/2003 - 05/2000)
3.	Boudreaux, Mary K: 3 articles (01/2007 - 12/2003)
4.	Seligsohn, Uri: 3 articles (04/2006 - 06/2003)
5.	Négrier, C: 3 articles (07/2004 - 05/2000)
6.	Nurden, A T: 3 articles (05/2004 - 10/2001)
7.	Nurden, Paquita: 3 articles (09/2002 - 03/2002)
8.	Combrié, Robert: 3 articles (09/2002 - 03/2002)
9.	Seligsohn, U: 3 articles (03/2002 - 03/2000)
10.	Fressinaud, E: 3 articles (10/2001 - 05/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Thrombasthenia:

1.	recombinant FVIIa (rFVIIa)IBA 03/01/2004 - "Recombinant factor VIIa (rFVIIa) is a safe and effective prohemostatic drug for patients with Glanzmann thrombasthenia (GT). " 09/01/2007 - "An established on-label indication for the use of rFVIIa is given in patients with Glanzmann thrombasthenia with refractoriness to transfusions of platelet concentrates. " 12/01/2006 - "Glanzmann thrombasthenia successfully operated for nasal deformation with recombinant factor VIIA." 10/01/2006 - "Use of rFVIIa in 4 children with Glanzmann thrombasthenia." 09/01/2006 - "Thromboelastographic evaluation of recombinant factor VIIa in a child with Glanzmann's thrombasthenia." Order ALL the reference details at left...
2.	Glycoproteins (Glycoprotein)IBA 06/01/2007 - "BACKGROUND: Glanzmann thrombasthenia (GT) is an exceedingly rare but well-defined inherited disorder of platelet function caused by a defect in the glycoprotein IIb/IIIa complex. " 05/01/2007 - "Three novel mutations in the glycoprotein IIb gene in a patient with type II Glanzmann thrombasthenia." 01/01/2007 - "A 10-base-pair deletion in the gene encoding platelet glycoprotein IIb associated with Glanzmann thrombasthenia in a horse." 07/01/2004 - "Glanzmann thrombasthenia associated with a 21-amino acid deletion (Leu817-Gln837) in glycoprotein IIb due to abnormal splicing in exon 25." 06/15/2003 - "Major mutations in calf-1 and calf-2 domains of glycoprotein IIb in patients with Glanzmann thrombasthenia enable GPIIb/IIIa complex formation, but impair its transport from the endoplasmic reticulum to the Golgi apparatus." Order ALL the reference details at left...
3.	AntibodiesIBA 09/01/2005 - "The three patients with Glanzmann's thrombasthenia developed anti-GPIIb/IIIa antibodies. " 01/01/1998 - "[Thrombasthenia with anti-platelet antibodies]" 06/01/1997 - "Pregnancy in a patient of Glanzmann's thrombasthenia with antiplatelet antibodies." 09/15/1994 - "To rule out a cross-reactivity of the two polyclonal antibodies with GPIIb/IIIa, we studied PECAM1 and CD9 expression on the platelets from a patient with type I Glanzmann's thrombasthenia whose platelets are devoid of GPIIb/IIIa. " 01/01/1994 - "Starting point is the peripheral blood from two patients with Glanzmann's thrombasthenia, an inherited disorder in which platelets lack these complexes, and where high titre antibodies to GPIIb-IIIa have formed following contact with normal platelets after transfusion and/or pregnancy. " Order ALL the reference details at left...
4.	Factor VIIa (Activated Factor VII)IBA 01/01/2006 - "Glanzmann's thrombasthenia treatment: a prospective observational registry on the use of recombinant human activated factor VII and other hemostatic agents." 12/01/2005 - "Recombinant activated factor VII is a safe and effective for the treatment and prevention of haemorrhage in haemophiliacs with circulating inhibitors to replacement factors, and patients with Glanzmann's thrombasthenia refractory to platelet transfusion. " 08/01/2006 - "Recombinant activated factor VII is approved for haemophiliacs with inhibitors and patients with thrombasthenia Glanzmann and factor VII deficiency. " 09/01/2003 - "The authors report here their experience with the use of factor VIIa, which may be useful for arresting bleeding in Glanzmann thrombasthenia." 02/01/2003 - "Recombinant activated factor VII combined with local measures in preventing bleeding from invasive dental procedures in patients with Glanzmann thrombasthenia." Order ALL the reference details at left...
5.	Platelet Glycoprotein GPIIb-IIIa ComplexIBA 10/01/2008 - "A beta3 Asp217-->Val substitution in a patient with variant Glanzmann Thrombasthenia severely affects integrin alphaIIBbeta3 functions." 10/15/2005 - "Therapeutic expression of the platelet-specific integrin, alphaIIbbeta3, in a murine model for Glanzmann thrombasthenia." 12/01/2002 - "To explore the possible role of a residual or variant alphaIIbbeta3 integrin (alphaIIbbeta3) in thrombogenesis, we used a new ex vivo perfusion chamber model to examine blood from patients with different subtypes of Glanzmann's thrombasthenia (GT). " 03/01/2002 - "Glanzmann thrombasthenia (GT) is an inherited disorder where an absence of platelet aggregation is associated with quantitative or qualitative abnormalities of the alphaIIbbeta3 integrin. " 03/01/2002 - "A novel 196Leu to Pro substitution in the beta3 subunit of the alphaIIbbeta3 integrin in a patient with a variant form of Glanzmann thrombasthenia." Order ALL the reference details at left...
6.	ThrombinIBA 03/01/1993 - "Furthermore, in platelets from patients with Glanzmann's thrombasthenia (which are deficient in the integrin receptor GPIIb-IIIa), pp60c-src was not translocated to the Triton-insoluble fraction, and there was a sustained increase in pp60c-src activity following thrombin treatment. " 08/01/1989 - "An increase of TSP after thrombin stimulation was observed on the surface of platelets of healthy controls and patients with Glanzmann's thrombasthenia (Type I). " 10/01/1982 - "In contrast, we now report that the specific binding of vWF to the thrombin-stimulated platelets was less than 20% of normal in three patients with Glanzmann thrombasthenia. " 05/15/1981 - "Glanzmann's thrombasthenia: deficient association of actin with the platelet membrane following thrombin-induced secretion." 02/01/1996 - "Binding sites of 125I-labelled LYP7, which was greatly increased on thrombin-stimulated (2 U/ml) washed platelets (10825 +/- 2886, mean +/- SD) Kd = 1.5 +/- 0.5 nM) compared to resting platelets (2801 +/- 1278, mean +/- SD) (Kd = 1.5 +/- 0.6 nM), was found to be normal on thrombin-stimulated platelets taken from a patient with grey platelet syndrome or a patient with Glanzmann thrombasthenia. " Order ALL the reference details at left...
7.	IsoantibodiesIBA 08/01/2006 - "Patients with Glanzmann thrombasthenia (GT) may form isoantibodies which induce refractoriness or inhibition of function of transfused platelets. " 06/01/2002 - "Correlation of activated clotting time (ACT) with rFVLLa infusion and clinical efficacy in a patient with Glanzmann thrombasthenia and platelet alloantibodies." 08/01/1979 - "The management of dental extractions in cases of thrombasthenia complicated by the development of isoantibodies to donor platelets." 12/01/2002 - "Our experience suggests that IA-PA, which restores the haemostatic efficacy of platelet transfusion, is a valuable therapeutic strategy in patients with Glanzmann's thrombasthenia and anti-GPIIb-IIIa isoantibodies." 03/01/1998 - "We report a case of a patient with Glanzmann's thrombasthenia and anti-GPIIb/IIIa alloantibodies who developed life-threatening and intractable bleeding from gastrointestinal telangiectatic lesions. " Order ALL the reference details at left...
8.	Tranexamic Acid (AMCA)FDA Link 04/01/2009 - "We suggest that rFVIIa at repeated doses of 80-100 microg/kg may be used effectively in patients with Glanzmann's thrombasthenia having excessive bleeding associated with hyperfibrinolysis after tonsillectomy in addition to tranexamic acid treatment." 01/01/2006 - "rFVIIa infusion after DDAVP administration could be useful in patients with Glanzmann's thrombasthenia in which DDAVP and tranexamic acid weren't always effective." Order ALL the reference details at left...
9.	Platelet Aggregation Inhibitors (Antiplatelet Drugs)IBA 10/15/2008 - "Research on alphaIIbbeta3 has been bidirectional, with basic insights resulting in improved Glanzmann thrombasthenia carrier detection and prenatal diagnosis, assays to identify single nucleotide polymorphisms responsible for alloimmune neonatal thrombocytopenia, and the development of alphaIIbbeta3 antagonists, the first rationally designed antiplatelet agents, to prevent and treat thrombotic cardiovascular disease. " 01/01/2005 - "Given that these characteristics are important determinants of the place of a treatment in bleeding disorders, recombinant factor VIIa provides a valuable treatment alternative in patients with haemophilia with inhibitors, platelet-refractory Glanzmann's thrombasthenia or congenital factor VII deficiency." Order ALL the reference details at left...
10.	Methotrexate (Mexate)FDA LinkGeneric 04/01/2006 - "Clinical remission of acquired thrombasthenia with low-dose methotrexate in a patient with systemic lupus erythematosus." Order ALL the reference details at left...

Therapies and Procedures

1.	Platelet Transfusion (Blood Platelet Transfusions) 01/01/1979 - "[Case of Glanzmann's thrombasthenia: a familial study and confirmation of the efficacy of platelet transfusions]" 03/01/1975 - "The response to platelet transfusions was studies in two patients with Glanzmann's thrombasthenia. " 08/01/2005 - "Isoantibody against GPIIb-IIIa complexes present on normal platelets can be observed in Glanzmann's thrombasthenia type I patients after platelet transfusion possibly leading to platelet transfusion refractoriness. " 10/01/2001 - "The place of this drug compared to platelet transfusion in the overall management of patients with Glanzmann thrombasthenia will need to be determined in relationship to a number of challenges and unresolved issues in the clinical care of these patients. " 11/01/1995 - "[Shear-induced platelet aggregation (SIPA) monitoring of hemostatic effect during platelet transfusion in a patient with Glanzmann's thrombasthenia: a comparison between SIPA and conventional platelet aggregation]" Order ALL the reference details at left...
2.	Tonsillectomy 01/01/1991 - "The transfusion requirements for a 6-year-old Glanzmann's thrombasthenia (GT) patient undergoing tonsillectomy and adenoidectomy were studied. " 04/01/2009 - "We report herein a 16-year-old boy with Glanzmann's thrombasthenia who did not respond to conservative treatment for excessive bleeding and hyperfibrinolysis after tonsillectomy and who was successfully treated with rFVIIa. " 04/01/2009 - "The use of recombinant FVIIa in a patient with Glanzmann thrombasthenia with uncontrolled bleeding after tonsillectomy." 04/01/2009 - "We suggest that rFVIIa at repeated doses of 80-100 microg/kg may be used effectively in patients with Glanzmann's thrombasthenia having excessive bleeding associated with hyperfibrinolysis after tonsillectomy in addition to tranexamic acid treatment." Order ALL the reference details at left...
3.	Cardiopulmonary Bypass (Heart-Lung Bypass) 08/01/2006 - "Perioperative management of a heterozygous carrier of Glanzmann's thrombasthenia submitted to coronary artery bypass grafting with cardiopulmonary bypass." 08/01/2006 - "We describe the perioperative management of a heterozygous carrier of Glanzmann's thrombasthenia submitted to cardiac surgery with cardiopulmonary bypass and the case was complicated by early excessive postoperative bleeding." 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). " Order ALL the reference details at left...
4.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 12/01/2003 - "Correction of a large animal model of type I Glanzmann's thrombasthenia by nonmyeloablative bone marrow transplantation." 02/01/2000 - "Bone marrow transplantation in severe Glanzmann's thrombasthenia with antiplatelet alloimmunization." 10/01/1997 - "We report the use of allegeneic bone marrow transplantation in a 5-year-old child with homozygous type I Glanzmann's thrombasthenia, using the patient's younger brother as marrow donor. " 10/01/1997 - "Sibling allogeneic bone marrow transplantation in a patient with type I Glanzmann's thrombasthenia." 07/01/1994 - "Bone marrow transplantation for Glanzmann's thrombasthenia." Order ALL the reference details at left...
5.	Transplants (Transplant) 09/01/2004 - "Less toxic RIC expands the potential application of stem cell transplants to patients with nonmalignant disorders of hematopoiesis, such as the severe form of Glanzmann thrombasthenia, who previously were not considered suitable candidates based on risk-benefit analysis. " 02/01/2000 - "We report the case of a 16-year-old girl, presenting with very severe type 1 Glanzmann's thrombasthenia, successfully treated with an HLA-identical sibling bone marrow transplant (BMT). " 05/01/2004 - "Acquired Glanzmann's thrombasthenia as part of multiple-autoantibody syndrome in a pediatric heart transplant patient." 12/01/2005 - "Sustained engraftment post bone marrow transplant despite anti-platelet antibodies in Glanzmann thrombasthenia." Order ALL the reference details at left...

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