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Glanzmann thrombasthenia
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Glanzmann thrombasthenia

Contributors: Nina Haghi MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Glanzmann thrombasthenia (GT) is an autosomal dominant coagulation disorder that results in spontaneous, prolonged, or heavy bleeding that can be life-threatening. It is caused by mutations in the ITGA2B or ITGB3 gene that result in defective or deficient platelet alpha IIb beta 3 integrin (formerly known as glycoprotein llb/llla), which is the platelet fibrinogen receptor. The functionality of this receptor is necessary for platelet aggregation. Acquired GT, on the other hand, is due to autoantibodies against alpha IIb beta 3 integrin.

Signs and symptoms include manifestations of mucocutaneous bleeding such as epistaxis, menorrhagia, petechiae, easy bruising, hematoma, and rarely intracranial hemorrhage or hematuria. Family history of bleeding is common.

Codes

ICD10CM:
D69.1 – Qualitative platelet defects

SNOMEDCT:
32942005 – Glanzmann Thrombasthenia

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Differential Diagnosis & Pitfalls

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Therapy

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References

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Last Reviewed:02/01/2021
Last Updated:01/16/2022
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Glanzmann thrombasthenia
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A medical illustration showing key findings of Glanzmann thrombasthenia : Bleeding time prolonged, Fibrinogen decreased, Ecchymosis, Epistaxis, Gingival hemorrhage, Menorrhagia
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