title: 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.
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan. yo-ogawa@gunma-u.ac.jp.
authors.forename: Yoshiyuki
authors.identifier: http://orcid.org/0000-0003-1459-3970
authors.initials: Y
authors.lastname: Ogawa
authors.affiliation: Department of Advanced Diagnosis, Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Kunishima
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Kunio
authors.initials: K
authors.lastname: Yanagisawa
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Osaki
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.|Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
authors.forename: Yuri
authors.initials: Y
authors.lastname: Uchiyama
authors.affiliation: Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
authors.forename: Naomichi
authors.initials: N
authors.lastname: Matsumoto
authors.affiliation: Department of Breast and Endocrine Surgery, Gunma University Graduate School of Medicine, Maebashi, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Tokiniwa
authors.affiliation: Department of Breast and Endocrine Surgery, Gunma University Graduate School of Medicine, Maebashi, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Horiguchi
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Nojima
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Handa
mesh_terms: D019468:Disease Management; D015942:Factor VIIa / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D006487:Hemostasis; D006801:Humans; D015409:Mastectomy, Radical / Q000379:methods*; D008875:Middle Aged; D008991:Monitoring, Physiologic / Q000379:methods; D019990:Perioperative Care / Q000379:methods*; D017713:Platelet Transfusion; D011994:Recombinant Proteins / Q000008:administration & dosage; D013915:Thrombasthenia / Q000097:blood* / Q000628:therapy
chemical_list: D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa
keywords: Anti-HLA alloantibodies; Flow cytometry; Glanzmann thrombasthenia; HLA-matched platelet transfusion; Recombinant human-activated factor VII