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:

Networked: 631 relevant articles (14 outcomes, 43 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1.	Nurden, P: 9 articles (06/2013 - 05/2000)
2.	Nurden, Alan T: 8 articles (10/2013 - 03/2002)
3.	Nurden, A T: 8 articles (06/2013 - 10/2001)
4.	Nurden, Paquita: 7 articles (08/2015 - 03/2002)
5.	Poon, Man-Chiu: 6 articles (08/2015 - 01/2006)
6.	Di Minno, Giovanni: 6 articles (08/2015 - 12/2004)
7.	Ghosh, Kanjaksha: 6 articles (01/2014 - 11/2002)
8.	Seligsohn, U: 6 articles (07/2012 - 03/2000)
9.	Seligsohn, Uri: 6 articles (05/2010 - 09/2002)
10.	Coller, Barry S: 6 articles (10/2008 - 09/2002)

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). " 08/01/2015 - "For all patients, regardless of platelet antibody or refractoriness status, recombinant factor VIIa, administered with or without platelets (±antifibrinolytics), provided effective hemostasis with a low frequency of adverse events in surgical procedures in Glanzmann thrombasthenia patients. " 06/01/2015 - "This article summarises published data on the mechanism of action and use of rFVIIa in these disorders from two international, prospective, observational registries: the Glanzmann's Thrombasthenia Registry (GTR) for GT; and the Seven Treatment Evaluation Registry (STER) for FVII CD. " 01/01/2015 - "Successful use of recombinant factor VIIa in a patient with acquired Glanzmann thrombasthenia." 06/01/2014 - "In European countries, rFVIIa is licensed for the above indications as well as for Glanzmann's thrombasthenia. "
2.	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. "
3.	Factor VIIa (Activated Factor VII)IBA 08/01/2015 - "To conclude, non-surgical bleeds were common and often severe in Glanzmann thrombasthenia; both platelets and recombinant activated factor VII appeared to be effective, and with good safety profiles, for the treatment of non-surgical bleeds. " 07/01/2014 - "Use of recombinant activated factor VII in patients with Glanzmann's thrombasthenia: a review of the literature." 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. "
4.	Glycoproteins (Glycoprotein)IBA 02/01/2014 - "Glanzmann thrombasthenia (GT) is an autosomal recessive disorder in which the platelet (PLT) glycoprotein IIb/IIIa complex is either deficient or dysfunctional. " 01/01/2014 - "Glanzmann thrombasthenia (GT) is a rare autosomal recessive disorder which deficiency of the platelet glycoprotein IIb - III a. " 09/01/2013 - "The platelet glycoprotein expression pattern in Iranian patients with Glanzmann's thrombasthenia was studied and the relationship between the platelet glycoprotein expression levels and clinical symptoms were investigated. " 01/01/2013 - "Glanzmann's thrombasthenia (GT) is an autosomal recessive disorder characterized by a lack of thrombocyte aggregation due to the absence of thrombocyte glycoproteins IIb and αIIbβ3. " 04/01/2012 - "Homozygous point mutations in platelet glycoprotein ITGA2B gene as cause of Glanzmann thrombasthenia in 2 families."
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."
6.	ThrombinFDA Link 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. "
7.	IsoantibodiesIBA 01/01/2011 - "Molecular analysis of a patient with type I Glanzmann thrombasthenia and clinical impact of the presence of anti-αIIbβ3 alloantibodies." 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."
8.	Tranexamic Acid (AMCA)FDA Link 01/01/2012 - "Giant abdominopelvic haematoma arising from ovulation in a Glanzmann's thrombasthenia patient with platelet refractoriness: treatment with surgery and intra-abdominal tranexamic acid." 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."
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. " 10/01/2012 - "Formation of low-calcium PS-positive platelets was promoted by platelet concentration increase or shaking and was decreased by integrin α(IIb)β(3) antagonists, platelet dilution, or in platelets from kindlin-3-deficient and Glanzmann thrombasthenia patients. " 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."
10.	Methotrexate (Mexate)FDA LinkGeneric 04/01/2006 - "Clinical remission of acquired thrombasthenia with low-dose methotrexate in a patient with systemic lupus erythematosus."

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. " 08/01/2015 - "Standard treatment for Glanzmann thrombasthenia is platelet transfusion. " 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. "
2.	Hematopoietic Stem Cell Transplantation 05/01/2011 - "Allogeneic hematopoietic stem cell transplantation in Glanzmann thrombasthenia complicated by platelet alloimmunization." 06/01/2009 - "We are the first to report the use of umbilical cord blood hematopoietic stem cell transplantation for Glanzmann thrombasthenia." 06/01/2009 - "Sustained engraftment and resolution of bleeding phenotype after unrelated cord blood hematopoietic stem cell transplantation for severe glanzmann thrombasthenia." 05/01/2011 - "For Thrombasthenia Glanzmann (GT) patients presenting with a severe clinical phenotype due to complete lack of thrombocyte function or increased titres of anti-platelet antibodies hematopoietic stem cell transplantation (SCT) is the only curative therapy. " 08/01/2011 - "To determine the safety and efficacy of off label usage of Recombinant activated factor VII (rFVIIa) in children with severe bleeding in non-hemophiliac children with diverse etiologies like leukemia, post hematopoietic stem cell transplantation, dengue shock syndrome and Glanzmann thrombasthenia. "
3.	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."
4.	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). "
5.	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."