Glanzmann's thrombasthenia history and symptoms
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Glanzmann's thrombasthenia |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2] Associate Editor(s)-in-Chief: Omer Kamal, M.D.[3]
Overview
Glanzmann's thrombasthenia is diagnosed at the neonatal age or early childhood, commonly before the age of 5 and the early manifestations are mostly easily bruising, mucocutaneous bleeding, epistaxis due to digital manipulation or a severe hemorrhage after a surgery, such as circumcision. The severity of the presenting symptoms has no known relation to the affected gene. However, mutations in the ITGB3 gene manifest bleeding more than the other gene. Symptoms of Glanzmann's thrombasthenia varies from a minor bruise to a life-threatening hemorrhage. It may include easily bruising (76.6%), nosebleeds that do not stop easily (62.5%), bleeding gums (56.4%), prolonged bleeding with minor injuries (47.2%), heavy menstrual bleeding, postpartum bleeding, gastrointestinal bleeding, heavy bleeding during and after surgery and bleeding into joints (rare).
History
Glanzmann's thrombasthenia is diagnosed at the neonatal age or early childhood, commonly before the age of 5 and the early manifestations are mostly easily bruising, mucocutaneous bleeding, epistaxis due to digital manipulation or a severe hemorrhage after a surgery, such as circumcision. The severity of the presenting symptoms has no known relation to the affected gene. However, mutations in the ITGB3 gene manifest bleeding more than the other gene.[1]
Symptoms
Symptoms of Glanzmann's thrombasthenia vary from a minor bruise to a life-threatening hemorrhage[1]. It may include any of the following manifestations:[1][2][3][4]
- Easily bruising (76.6%)
- Nosebleeds that do not stop easily (62.5%)
- Bleeding gums (56.4%)
- Prolonged bleeding with minor injuries (47.2%)
- Heavy menstrual bleeding
- Postpartum bleeding
- Gastrointestinal bleeding
- Heavy bleeding during and after surgery
- Bleeding into joints (rare) [5]
References
- ↑ 1.0 1.1 1.2 Bakdash S, Lyons JM, Bastacky SI, Pezzone MA, McGee JB, Schoen RE, Regueiro M, Lee KK, Bontempo FA (May 2008). "Management of persistent gastric bleeding in a patient with Glanzmann's thrombasthenia". Am. J. Hematol. 83 (5): 411–5. doi:10.1002/ajh.21122. PMID 18186523.
- ↑ George JN, Caen JP, Nurden AT (April 1990). "Glanzmann's thrombasthenia: the spectrum of clinical disease". Blood. 75 (7): 1383–95. PMID 2180491.
- ↑ Toogeh G, Sharifian R, Lak M, Safaee R, Artoni A, Peyvandi F (October 2004). "Presentation and pattern of symptoms in 382 patients with Glanzmann thrombasthenia in Iran". Am. J. Hematol. 77 (2): 198–9. doi:10.1002/ajh.20159. PMID 15389911.
- ↑ Nurden AT, Fiore M, Nurden P, Pillois X (December 2011). "Glanzmann thrombasthenia: a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models". Blood. 118 (23): 5996–6005. doi:10.1182/blood-2011-07-365635. PMID 21917754.
- ↑ Iqbal I, Farhan S, Ahmed N (2016). "Glanzmann Thrombasthenia: A Clinicopathological Profile". J Coll Physicians Surg Pak. 26 (8): 647–50. doi:2396 Check
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