Korean Journal of Anesthesiology  2009;57(4):507-510

doi:10.4097/kjae.2009.57.4.507

Anesthetic experience for orthopedic surgery on a patient with Glanzmann's thrombasthenia refractory to platelet transfusion: A case report.

Jung Bok PARK 1 ; Yang Sik SHIN ; Soo Hwan KIM

Affiliations

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Keywords

General anesthesia; Glanzmann's thrombasthenia; Platelet transfusion

Country

Republic of Korea

Language

Korean

Abstract

Glanzmann's thrombasthenia is an autosomal recessive bleeding disorder caused by qualitative or quantitative abnormalities of the platelet glycoprotein IIb/IIIa (GP IIb/IIIa), which can lead to excessive bleeding. Glanzmann thrombasthenia is associated with clinical variability, with some patients only having minimal bruising and others having frequent, severe and potentially fatal hemorrhages. Platelet transfusions, which used to be the standard treatment, may lead to the development of antibodies to HLA and/or GPIIb/IIIa, thereby rendering future transfusions ineffective. Glanzmann's thrombasthenia can be a severe hemorrhagic disease; however, the prognosis is excellent with careful supportive care. In this case, administering allogenic plateletpheresis to patients with Glanzmann's thrombasthenia who were refractory to platelet transfusions was found to be successful during bone surgeries.