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Successful management of perioperative hemostasis in a patient with Glanzmann thrombasthenia who underwent a right total mastectomy.

Abstract：

:Perioperative hemostatic management is a challenge in patients with Glanzmann thrombasthenia (GT). The standard means of preventing surgical bleeding in GT patients is platelet transfusion. However, GT patients often possess alloantibodies against GPIIb/IIIa and/or HLA, which cause resistance to platelet transfusion. HLA-matched platelet transfusion, plasmapheresis, or recombinant human-activated factor VII (rFVIIa) are alternative interventions in such cases. Monitoring of hemostasis is also critical in the management of GT patients who undergo surgery. Here, we report the case of a 56-year-old female GT patient with anti-HLA antibodies, who underwent a right total mastectomy without significant blood loss under HLA-matched platelet transfusion. Bleeding at the surgical site, which occurred on the 18th postoperative day, was successfully treated by immediate bolus administration of rFVIIa and subsequent HLA-matched platelet transfusion. The perioperative hemostatic state was monitored in combination with bleeding time, platelet aggregation assay, and flow cytometric analysis of GPIIb/IIIa expression. Although a flow cytometric analysis is not a functional assay, it enabled the estimation of transfused platelet counts, and helped to inform the decision regarding whether to perform the surgery. Thus, perioperative hemostasis was successfully managed in our GT patient by HLA-matched platelet transfusion, rFVIIa administration, and the close monitoring of hemostasis.

journal_name

Int J Hematol

journal_title

International journal of hematology

authors

Ogawa Y,Kunishima S,Yanagisawa K,Osaki Y,Uchiyama Y,Matsumoto N,Tokiniwa H,Horiguchi J,Nojima Y,Handa H

doi

10.1007/s12185-016-2096-x

subject

Has Abstract

pub_date

2017-02-01 00:00:00

pages

221-225

issue

2

eissn

0925-5710

issn

1865-3774

pii

10.1007/s12185-016-2096-x

journal_volume

105

pub_type

杂志文章

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