Glanzmann's thrombasthenia causes: Difference between revisions
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# myelodysplastic syndrome | # myelodysplastic syndrome | ||
# immune thrombocytopenic purpura (ITP) | # immune thrombocytopenic purpura (ITP) | ||
* Autoimmune diseases, such as lupus | * 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> | ||
* Drugs : anti-thrombotic drugs use , like abciximab, eptifibatide, and tirofiban which all antagonize αIIbβ3 | * 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> | * 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> |
Revision as of 05:08, 19 July 2018
Glanzmann's thrombasthenia |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Glanzmann's thrombasthenia can be inherited in an autosomal recessive manner or acquired as an autoimmune disorder.[1]
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
In the hereditary type of Glanzmann's thrombasthenia GPIIb/IIIa (ITG αIIbβ3) is qualitative or quantitative disordered .
The autoantibodies production is the main cause of acquired Glanzmann's thrombasthenia. It can be produced in the of following conditions:
- Hematologic disorders and malignancies, such as :
- acute lymphoblastic leukemia,
- non-Hodgkin’s lymphoma
- multiple myeloma
- hairy cell leukemia
- myelodysplastic syndrome
- immune thrombocytopenic purpura (ITP)
- Autoimmune diseases, such as lupus [2]
- Drugs : anti-thrombotic drugs use , like abciximab, eptifibatide, and tirofiban which all antagonize αIIbβ3
- platelet transfusions.[3]
References
- ↑ 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.
- ↑ 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.
- ↑ 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.