Glanzmann's thrombasthenia medical therapy
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
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.