Glanzmann's thrombasthenia medical therapy: Difference between revisions
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==Overview== | ==Overview== | ||
The treatment of [[bleeding]] episodes in patients with [[glanzmann's thrombasthenia]] includes local measures with or without anti-fibrinolytic therapy first, followed by [[platelet]] transfusion, and rFVIIa if bleeding persists. However, The majority of cases of [[glanzmann's thrombasthenia]] are self-limited and only require supportive care. Other options include [[desmopressin]] (DDAVP) which increases in [[plasma]], the [[tissue plasminogen activator]] (TPA),[[FVIII]] and [[VWF]], but it has no significant effect on [[platelet]] disorders, rFVIIa: Manages [[bleeding]] in most patients with [[glanzmann's thrombasthenia]], [[rituximab]], [[bevacizumab]],[[Hematopoietic stem cell transplantation|hematopoietic stem cell transplantation and]] [[gene therapy]]. | |||
==Medical Therapy== | ==Medical Therapy== | ||
The treatment of bleeding episodes in patients with | The treatment of [[bleeding]] episodes in patients with [[glanzmann's thrombasthenia]] includes local measures with or without anti-fibrinolytic therapy first, followed by [[platelet]] transfusion, and rFVIIa if bleeding persists. However, The majority of cases of [[glanzmann's thrombasthenia]] are self-limited and only require supportive care.<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> Other options include: | ||
* Desmopressin (DDAVP): Increases in plasma the tissue plasminogen activator (TPA),FVIII and VWF, but it has no significant effect on platelet disorders<ref name="pmid22781097">{{cite journal |vauthors=Fiore M, Nurden AT, Nurden P, Seligsohn U |title=Clinical utility gene card for: Glanzmann thrombasthenia |journal=Eur. J. Hum. Genet. |volume=20 |issue=10 |pages= |date=October 2012 |pmid=22781097 |pmc=3449071 |doi=10.1038/ejhg.2012.151 |url=}}</ref> | * [[Desmopressin]] (DDAVP): Increases in [[plasma]], the [[tissue plasminogen activator]] (TPA),[[FVIII]] and [[VWF]], but it has no significant effect on [[platelet]] disorders<ref name="pmid22781097">{{cite journal |vauthors=Fiore M, Nurden AT, Nurden P, Seligsohn U |title=Clinical utility gene card for: Glanzmann thrombasthenia |journal=Eur. J. Hum. Genet. |volume=20 |issue=10 |pages= |date=October 2012 |pmid=22781097 |pmc=3449071 |doi=10.1038/ejhg.2012.151 |url=}}</ref> | ||
*rFVIIa: Manages bleeding in most patients with glanzmann's thrombasthenia <ref name="pmid12472566">{{cite journal |vauthors=Stevens RF, Meyer S |title=Fanconi and Glanzmann: the men and their works |journal=Br. J. Haematol. |volume=119 |issue=4 |pages=901–4 |date=December 2002 |pmid=12472566 |doi= |url=}}</ref><ref name="pmid11897046">{{cite journal |vauthors=Nurden AT, Ruan J, Pasquet JM, Gauthier B, Combrié R, Kunicki T, Nurden P |title=A novel 196Leu to Pro substitution in the beta3 subunit of the alphaIIbbeta3 integrin in a patient with a variant form of Glanzmann thrombasthenia |journal=Platelets |volume=13 |issue=2 |pages=101–11 |date=March 2002 |pmid=11897046 |doi=10.1080/09537100220122466 |url=}}</ref> | *rFVIIa: Manages [[bleeding]] in most patients with [[glanzmann's thrombasthenia]] <ref name="pmid12472566">{{cite journal |vauthors=Stevens RF, Meyer S |title=Fanconi and Glanzmann: the men and their works |journal=Br. J. Haematol. |volume=119 |issue=4 |pages=901–4 |date=December 2002 |pmid=12472566 |doi= |url=}}</ref><ref name="pmid11897046">{{cite journal |vauthors=Nurden AT, Ruan J, Pasquet JM, Gauthier B, Combrié R, Kunicki T, Nurden P |title=A novel 196Leu to Pro substitution in the beta3 subunit of the alphaIIbbeta3 integrin in a patient with a variant form of Glanzmann thrombasthenia |journal=Platelets |volume=13 |issue=2 |pages=101–11 |date=March 2002 |pmid=11897046 |doi=10.1080/09537100220122466 |url=}}</ref> | ||
* Rituximab: Anti-CD20 <ref name="pmid11776310">{{cite journal |vauthors=Morel-Kopp MC, Melchior C, Chen P, Ammerlaan W, Lecompte T, Kaplan C, Kieffer N |title=A naturally occurring point mutation in the beta3 integrin MIDAS-like domain affects differently alphavbeta3 and alphaIIIbbeta3 receptor function |journal=Thromb. Haemost. |volume=86 |issue=6 |pages=1425–34 |date=December 2001 |pmid=11776310 |doi= |url=}}</ref> | * [[Rituximab]]: Anti-[[CD20]] <ref name="pmid11776310">{{cite journal |vauthors=Morel-Kopp MC, Melchior C, Chen P, Ammerlaan W, Lecompte T, Kaplan C, Kieffer N |title=A naturally occurring point mutation in the beta3 integrin MIDAS-like domain affects differently alphavbeta3 and alphaIIIbbeta3 receptor function |journal=Thromb. Haemost. |volume=86 |issue=6 |pages=1425–34 |date=December 2001 |pmid=11776310 |doi= |url=}}</ref> | ||
* Bevacizumab: Anti-VEGF antibody<ref name="pmid12472566">{{cite journal |vauthors=Stevens RF, Meyer S |title=Fanconi and Glanzmann: the men and their works |journal=Br. J. Haematol. |volume=119 |issue=4 |pages=901–4 |date=December 2002 |pmid=12472566 |doi= |url=}}</ref> | * [[Bevacizumab]]: Anti-[[VEGF]] antibody<ref name="pmid12472566">{{cite journal |vauthors=Stevens RF, Meyer S |title=Fanconi and Glanzmann: the men and their works |journal=Br. J. Haematol. |volume=119 |issue=4 |pages=901–4 |date=December 2002 |pmid=12472566 |doi= |url=}}</ref> | ||
* Hematopoietic stem cell transplantation<ref name="pmid22781097">{{cite journal |vauthors=Fiore M, Nurden AT, Nurden P, Seligsohn U |title=Clinical utility gene card for: Glanzmann thrombasthenia |journal=Eur. J. Hum. Genet. |volume=20 |issue=10 |pages= |date=October 2012 |pmid=22781097 |pmc=3449071 |doi=10.1038/ejhg.2012.151 |url=}}</ref> | * [[Hematopoietic stem cell transplantation]]<ref name="pmid22781097">{{cite journal |vauthors=Fiore M, Nurden AT, Nurden P, Seligsohn U |title=Clinical utility gene card for: Glanzmann thrombasthenia |journal=Eur. J. Hum. Genet. |volume=20 |issue=10 |pages= |date=October 2012 |pmid=22781097 |pmc=3449071 |doi=10.1038/ejhg.2012.151 |url=}}</ref> | ||
* Gene therapy<ref name="pmid261854782">{{cite journal |vauthors=Solh T, Botsford A, Solh M |title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options |journal=J Blood Med |volume=6 |issue= |pages=219–27 |date=2015 |pmid=26185478 |pmc=4501245 |doi=10.2147/JBM.S71319 |url=}}</ref><ref name="pmid22781097">{{cite journal |vauthors=Fiore M, Nurden AT, Nurden P, Seligsohn U |title=Clinical utility gene card for: Glanzmann thrombasthenia |journal=Eur. J. Hum. Genet. |volume=20 |issue=10 |pages= |date=October 2012 |pmid=22781097 |pmc=3449071 |doi=10.1038/ejhg.2012.151 |url=}}</ref> | * [[Gene therapy]]<ref name="pmid261854782">{{cite journal |vauthors=Solh T, Botsford A, Solh M |title=Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options |journal=J Blood Med |volume=6 |issue= |pages=219–27 |date=2015 |pmid=26185478 |pmc=4501245 |doi=10.2147/JBM.S71319 |url=}}</ref><ref name="pmid22781097">{{cite journal |vauthors=Fiore M, Nurden AT, Nurden P, Seligsohn U |title=Clinical utility gene card for: Glanzmann thrombasthenia |journal=Eur. J. Hum. Genet. |volume=20 |issue=10 |pages= |date=October 2012 |pmid=22781097 |pmc=3449071 |doi=10.1038/ejhg.2012.151 |url=}}</ref> | ||
==References== | ==References== | ||
Revision as of 20:47, 12 December 2018
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Glanzmann's thrombasthenia |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Omer Kamal, M.D.[2]
Overview
The treatment of bleeding episodes in patients with glanzmann's thrombasthenia includes local measures with or without anti-fibrinolytic therapy first, followed by platelet transfusion, and rFVIIa if bleeding persists. However, The majority of cases of glanzmann's thrombasthenia are self-limited and only require supportive care. Other options include desmopressin (DDAVP) which increases in plasma, the tissue plasminogen activator (TPA),FVIII and VWF, but it has no significant effect on platelet disorders, rFVIIa: Manages bleeding in most patients with glanzmann's thrombasthenia, rituximab, bevacizumab,hematopoietic stem cell transplantation and gene therapy.
Medical Therapy
The treatment of bleeding episodes in patients with glanzmann's thrombasthenia includes local measures with or without anti-fibrinolytic therapy first, followed by platelet transfusion, and rFVIIa if bleeding persists. However, The majority of cases of glanzmann's thrombasthenia are self-limited and only require supportive care.[1] Other options include:
- Desmopressin (DDAVP): Increases in plasma, the tissue plasminogen activator (TPA),FVIII and VWF, but it has no significant effect on platelet disorders[2]
- rFVIIa: Manages bleeding in most patients with glanzmann's thrombasthenia [3][4]
- Rituximab: Anti-CD20 [5]
- Bevacizumab: Anti-VEGF antibody[3]
- Hematopoietic stem cell transplantation[2]
- Gene therapy[6][2]
References
- ↑ 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.
- ↑ 2.0 2.1 2.2 Fiore M, Nurden AT, Nurden P, Seligsohn U (October 2012). "Clinical utility gene card for: Glanzmann thrombasthenia". Eur. J. Hum. Genet. 20 (10). doi:10.1038/ejhg.2012.151. PMC 3449071. PMID 22781097.
- ↑ 3.0 3.1 Stevens RF, Meyer S (December 2002). "Fanconi and Glanzmann: the men and their works". Br. J. Haematol. 119 (4): 901–4. PMID 12472566.
- ↑ Nurden AT, Ruan J, Pasquet JM, Gauthier B, Combrié R, Kunicki T, Nurden P (March 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". Platelets. 13 (2): 101–11. doi:10.1080/09537100220122466. PMID 11897046.
- ↑ Morel-Kopp MC, Melchior C, Chen P, Ammerlaan W, Lecompte T, Kaplan C, Kieffer N (December 2001). "A naturally occurring point mutation in the beta3 integrin MIDAS-like domain affects differently alphavbeta3 and alphaIIIbbeta3 receptor function". Thromb. Haemost. 86 (6): 1425–34. PMID 11776310.
- ↑ 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.