Glanzmann's thrombasthenia causes: Difference between revisions

Jump to navigation Jump to search
No edit summary
No edit summary
Line 5: Line 5:


==Overview==
==Overview==
Glanzmann's thrombasthenia can be inherited in an [[autosomal]] [[recessive]] manner or acquired as an [[autoimmune disorder]].<ref>Tholouli E, Hay CR, O'Gorman P, Makris M. Acquired Glanzmann's thrombasthenia without thrombocytopenia: a severe acquired autoimmune bleeding disorder. Br J Haematol. 2004 Oct;127(2):209-13. PMID 15461628.</ref>
Glanzmann's thrombasthenia can be inherited in an [[autosomal]] [[recessive]] manner or acquired as an [[autoimmune disorder]]. In the hereditary type of [[Glanzmann's thrombasthenia]] GPIIb/IIIa (ITG αIIbβ3) is qualitative or quantitative disorder. The [[autoantibodies]] production is the main cause of acquired [[Glanzmann's thrombasthenia]] It can be produced in the [[Acute lymphoblastic leukemia]], [[Non-Hodgkin lymphoma]], [[Multiple myeloma]], [[Hairy cell leukemia]], [[Myelodysplastic syndrome]], [[Idiopathic thrombocytopenic purpura|Immune thrombocytopenic purpura]] (ITP), [[Pregnancy]], [[Autoimmune disease|Autoimmune diseases]] (eg, [[systemic lupus erythematosus]], [[Immune]] [[thrombocytopenia]]),Anti-thrombotic drugs use , like [[abciximab]], [[eptifibatide]], and [[tirofiban]] which all antagonize αIIbβ3 and [[Platelet transfusions]].  
 
In the hereditary type of GT there is qualitative or quantitative defect in GPIIb/IIIa (ITG αIIbβ3). GPIIb/IIIa is a receptor on platelet surface, which takes part in platelet aggregation and adhesion, regulation of cell migration and thrombus formation, and when it's dysfunction lead to reduced clot retraction.
 
Autoantibodies production is the main cause of acquired Glanzmann's thrombasthenia. Isoantibodies are being produced in multiple conditions such as, hematologic disorders and malignancies acute lymphoblastic leukemia,non-Hodgkin’s lymphoma, multiple myeloma, hairy cell leukemia,myelodysplastic syndrome and ITP. Autoimmune diseases, such as lupus (Blickstein et al).Drugs like anti-thrombotic drugs use , like abciximab, eptifibatide, and tirofiban which all antagonize αIIbβ3. And platelet transfusions


==Causes==
==Causes==
In the hereditary type of Glanzmann's thrombasthenia GPIIb/IIIa (ITG αIIbβ3) is qualitative or quantitative disordered .
In the hereditary type of [[Glanzmann's thrombasthenia]] GPIIb/IIIa (ITG αIIbβ3) is qualitative or quantitative disorder. The [[autoantibodies]] production is the main cause of acquired [[Glanzmann's thrombasthenia]] It can be produced in the of following conditions:
 
* [[Acute lymphoblastic leukemia]]
The autoantibodies production is the main cause of acquired Glanzmann's thrombasthenia. It can be produced in the of following conditions:
* [[Non-Hodgkin lymphoma]]
* Hematologic disorders and malignancies, such as :
* [[Multiple myeloma]]
# acute lymphoblastic leukemia,
* [[Hairy cell leukemia]]
# non-Hodgkin’s lymphoma
* [[Myelodysplastic syndrome]]
# multiple myeloma
* [[Idiopathic thrombocytopenic purpura|Immune thrombocytopenic purpura]] (ITP)
# hairy cell leukemia
* [[Pregnancy]]
# myelodysplastic syndrome
* [[Autoimmune disease|Autoimmune diseases]] (eg, [[systemic lupus erythematosus]], [[Immune]] [[thrombocytopenia]])<ref name="pmid24979837">{{cite journal| author=Blickstein D, Dardik R, Rosenthal E, Lahav J, Molad Y, Inbal A| title=Acquired thrombasthenia due to inhibitory effect of glycoprotein IIbIIIa autoantibodies. | journal=Isr Med Assoc J | year= 2014 | volume= 16 | issue= 5 | pages= 307-10 | pmid=24979837 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24979837  }}</ref>
# immune thrombocytopenic purpura (ITP)
* Drugs : Anti-thrombotic drugs use , like [[abciximab]], [[eptifibatide]], and [[tirofiban]] which all antagonize αIIbβ3
* Autoimmune diseases, such as lupus <ref name="pmid24979837">{{cite journal| author=Blickstein D, Dardik R, Rosenthal E, Lahav J, Molad Y, Inbal A| title=Acquired thrombasthenia due to inhibitory effect of glycoprotein IIbIIIa autoantibodies. | journal=Isr Med Assoc J | year= 2014 | volume= 16 | issue= 5 | pages= 307-10 | pmid=24979837 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24979837  }}</ref>
* [[Platelet transfusions]]<ref name="pmid26185478">{{cite journal| author=Solh T, Botsford A, Solh M| title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. | journal=J Blood Med | year= 2015 | volume= 6 | issue=  | pages= 219-27 | pmid=26185478 | doi=10.2147/JBM.S71319 | pmc=4501245 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26185478  }}</ref>
* Drugs : anti-thrombotic drugs use , like abciximab, eptifibatide, and tirofiban which all antagonize αIIbβ3
* platelet transfusions.<ref name="pmid26185478">{{cite journal| author=Solh T, Botsford A, Solh M| title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. | journal=J Blood Med | year= 2015 | volume= 6 | issue=  | pages= 219-27 | pmid=26185478 | doi=10.2147/JBM.S71319 | pmc=4501245 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26185478  }}</ref>
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Revision as of 18:10, 13 November 2018

Glanzmann's thrombasthenia

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Glanzmann's thrombasthenia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

Echocardiography and Ultrasound

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Glanzmann's thrombasthenia causes On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Glanzmann's thrombasthenia causes

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Glanzmann's thrombasthenia causes

CDC on Glanzmann's thrombasthenia causes

Glanzmann's thrombasthenia causes in the news

Blogs on Glanzmann's thrombasthenia causes

Directions to Hospitals Treating Type page name here

Risk calculators and risk factors for Glanzmann's thrombasthenia causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Niyousha Danesh, MD-MPH

Overview

Glanzmann's thrombasthenia can be inherited in an autosomal recessive manner or acquired as an autoimmune disorder. In the hereditary type of Glanzmann's thrombasthenia GPIIb/IIIa (ITG αIIbβ3) is qualitative or quantitative disorder. The autoantibodies production is the main cause of acquired Glanzmann's thrombasthenia It can be produced in the Acute lymphoblastic leukemia, Non-Hodgkin lymphoma, Multiple myeloma, Hairy cell leukemia, Myelodysplastic syndrome, Immune thrombocytopenic purpura (ITP), Pregnancy, Autoimmune diseases (eg, systemic lupus erythematosus, Immune thrombocytopenia),Anti-thrombotic drugs use , like abciximab, eptifibatide, and tirofiban which all antagonize αIIbβ3 and Platelet transfusions.

Causes

In the hereditary type of Glanzmann's thrombasthenia GPIIb/IIIa (ITG αIIbβ3) is qualitative or quantitative disorder. The autoantibodies production is the main cause of acquired Glanzmann's thrombasthenia It can be produced in the of following conditions:

References

  1. Blickstein D, Dardik R, Rosenthal E, Lahav J, Molad Y, Inbal A (2014). "Acquired thrombasthenia due to inhibitory effect of glycoprotein IIbIIIa autoantibodies". Isr Med Assoc J. 16 (5): 307–10. PMID 24979837.
  2. Solh T, Botsford A, Solh M (2015). "Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options". J Blood Med. 6: 219–27. doi:10.2147/JBM.S71319. PMC 4501245. PMID 26185478.