Management of Surgeries in Glanzmann Thrombastenia: Single Center Experience

INTRODUCTION: Glanzmann thrombasthenia (GT) is a rare thrombocyte aggregation defect and there are no guidelines for supporting surgical hemostasis in patients with GT. The aim of the present study is to share the experiences in bleeding control of surgical interventions of patients with GT.
METHODS: In this retrospective study, patients with a diagnosis of GT, followed-up between 1999 and 2000 and underwent surgical procedure were analyzed. Patients’ age at diagnosis, bleeding characteristics, surgical procedures, hemostatic treatments, and bleeding complications of surgery were analyzed.
RESULTS: Twenty-three surgical procedures were performed in 13 patients. Thirteen (56.5%) of these procedures were tooth extractions. Thrombocyte concentrate, and oral tranexamic acid were started before tooth extractions and oral treatment was continued for 5 days but in three of these procedures bleeding control was poor after the procedure. In two of these procedures, bleeding was controlled with recombinant factor VIIa (rFVIIa). In bronchoscopy, varicocele, circumcision, thyroglosal cyst operations and molar tooth extractions, rFVIIa was used alone or in addition and bleeding control was achieved. In four patients with history of surgical interventions performed before GT diagnosis, bleeding complications were reported whereas bleeding control was good in a cesarean operation performed without any hemostatic agent.
DISCUSSION AND CONCLUSION: Patients with suspected bleeding defect or with bleeding defect must be referred to the hematologist. Abnormal platelet clumping in peripheral smear performed from the fingertip, prolonged bleeding time suggest GT. Surgical procedures in patients with GT can be safely performed with collaboration of a hematologist by administering tranexamic acid, thrombocyte suspension and rVIIa.

Glanzmann Trombastenisinde Cerrahi Giriþimlerin Yönetimi: Tek Merkez Deneyimi

GÝRÝÞ ve AMAÇ: Glanzmann trombastenisi (GT) nadir görülen trombosit agregasyon kusurudur ve GT tanýlý hastalarýn cerrahi giriþimlerinde uygulanacak hemostaz desteði ile ilgili rehberler bulunmamaktadýr. Bu çalýþmada GT tanýsý ile takip edilen hastalarda uygulanan cerrahi giriþimlerde kanama kontrolü ile ilgili deneyimlerin paylaþýlmasý amaçlandý.
YÖNTEM ve GEREÇLER: 13 Geriye dönük yapýlan bu çalýþmada 1999-2020 yýllarý arasýnda GT tanýsý alarak izlenen hastalara uygulanan cerrahi iþlemler deðerlendirildi Hastalarýn taný yaþlarý, kanama þekilleri, hastalara uygulanan cerrahi iþlemler ve cerrahi iþlemlerde uygulanan tedaviler ile cerrahi iliþkili kanamalar incelendi.
BULGULAR: Çalýþmada 13 hastaya, 23 cerrahi iþlem uygulandý. Ýþlemlerin 13’ü (%56,5) diþ çekimiydi. Tanýsý bilinen hastalarýn diþ çekimlerinde, iþlem öncesi trombosit süspansiyonu ve oral traneksamik asit baþlandý, oral tedavi 5 gün sürdürüldü. Bronkoskopi, varikosel, sünnet, tiroglosal kist operasyonu ve azý diþi çekimlerinde ise bu tedavilere ilave olarak veya tek baþýna rekombinant Faktör VIIa (rFVIIa) kullanýldý ve kanama kontrolü saðlandý. Üç hastanýn diþ çekimi öncesi traneksamit asit ve trombosit süspansiyonu verilmesine karþýn iþlem sonrasýnda kanama gözlendi. Bu iþlemlerin ikisinde rFVIIa da tedaviye eklenerek kanama kontrolü saðlandý. Taný öncesi, herhangi bir hemostatik ajan verilmeden cerrahi giriþim uygulandýðý belirtilen dört hastada kanama komplikasyonu geliþtiði; bir hastada ise hemostatik ajan verilmemesine karþýn sezaryen operasyonunda kanama kontrolünün iyi olduðu öðrenildi.
TARTIÞMA ve SONUÇ: Kanama açýsýndan þüpheli öyküsü olan veya kanama kusuru saptanmýþ hastalar cerrahi öncesi hematoloji uzmanýna yönlendirilmelidir. Parmak ucundan yapýlacak periferik yaymada trombosit kümelenmesinin görülmemesi ve kanama zamanýnýn uzun olmasý GT tanýsýna yönlendirir. GT tanýlý hastalarda cerrahi iþlemler hematoloji uzmaný iþ birliðiyle, traneksamik asit, trombosit süspansiyonu ve rFVIIa uygulanarak güvenle yapýlabilir.