Berrin GÜNAYDIN, Ahmet ERDEM, Atilla ALTINSOY, Rauf HAZNEDAR

Glanzman trombastenili gebede sezaryen operasyonunda rekombinant aktive faktör VII kullanımı

Glanzman Trombastenisi (GT), trombosit yüzeyinde glikoprotein IIb/IIIa eksikliği ya da fonksiyon bozukluğu ile karakterize konjenital bir kanama bozukluğudur. Son yıllarda GT’li trombosit transfüzyonuna refrakter olgularda rekombinant aktive Faktör VII (rFVIIa) başarıyla kullanılmıştır. Bu olgu sunumunda çocukluk çağında kullanılmış olan trombositlere karşı gelişen alloim- munizasyon nedeniyle rFVIIa kullanımının önemi sunuldu. Termde GT’li gebeye genel anestezi altında sezaryen planlandı. Hematologlar hastaya preoperatif olarak 90 µg kg-1 (4,5 KIU kg-1 veya 240 KIU) rFVIIa uygulanmasını ve postoperatif dönemde de devam edilmesini önerdi. Preoperatif tam kan sayımı (hematokrit, beyaz küre, trombosit), koagülasyon testleri (INR, PT, aPTT), faktör VII (prokonvertin) ve D-dimer düzeyleri normal sınırlar içindeydi. Fakat fibrinojen düzeyi yüksek, kanama zamanı uzun, trombosit agregasyon testlerinden ADP ve kollajene yanıt düşük, ristosetin 1 mg’a ise normal yanıt vardı. Tiyopental ve rokuronyum ile anestezi indüksiyonunu takiben endotrakeal entü- basyon gerçekleştirildi ve %50 azot-oksijenle birlikte propofol infüzyonuyla anestezi idame ettirildi. Göbek kordonu klemplendikten sonra 10 IU oksitosin iv infüzyonla ve 0.2 mg metil ergometrin im olarak uygulandı. Sonuç olarak GT’li bu gebe olguda preoperatif dönemde başlanıp, postoperatif dönemde de sürdürülen rFVIIa uygulamasıyla gerek cerrahi gerekse anestezi sorunsuz tamamlandı. Olgunun postpartum geç dönemdeki vajinal kanaması, yeniden rFVIIa uygulamasına gerek kalmadan uterotonik ve antifibrinolitik tedaviyle kontrol altına alındı. Böylece nadir gözlenen GT’li bir gebede sezaryen operasyonunun genel anesteziyle sorunsuz şekilde gerçekleştirilmesinde rFVIIa kullanımının önemi vurgulandı.

Anahtar Kelimeler:

Erişkin, Kadın, Anestezi, genel, Sezaryen, Trombasteni, Trombositopeni, Faktör VIIa

The use of recombinat activated factor VII in a perturient with Glanzmann thrombasthenia during cesarean section

Glanzmann thrombasthenia (GT) is a congenital bleeding disorder characterized by deficiency or dysfunction of platelet surface glycoprotein IIb/IIIa. Recombinant activated factor VII (rFVIIa) has been successfully used in cases with GT refractory to platelet transfusion lately. The importance of rFVIIa use because of the alloimmunization developed due to previous platelet transfusion during childhood of the patient was presented in this case report. Cesarean section under general anesthesia was planned for the term parturient with GT. Hematologists suggested to administer 90 µg kg-1 (4,5 KIU kg-1 or 240 KIU) rFVIIa preoperatively and to continue in the postoperative period. Preoperative complete blood count (hematocrit, white blood cell, platelet), coagulation tests (INR, PT, aPTT), factor VII (proconvertin) and D-dimer levels were within normal limits. However, fibrinogen level was high, bleeding time was long and response to ADP and collagen were low while response to ristocetin 1 mg was normal in the platelet aggregation tests. After anesthesia induction with thiopentone and rocuronium, endotracheal intubation was performed and anesthesia was maintained in 50% nitrous oxide-oxygen mixture with propofol infusion. 10 IU of oxytocin was given by iv infusion and intramuscular ergometrine was administered following umbilical cord clamping. In conclusion either surgery or anesthesia has been completed uneventfully because of starting rFVIIa administration preoperatively and continuing in the postoperative period as well in the present parturient with GT. The importance of rFVIIa administration has been emphasized in a parturient with GT underwent an uneventful cesarean section under general anesthesia.

Keywords:

Adult, Female, Anesthesia, General, Cesarean Section, Thrombasthenia, Thrombocytopenia, Factor VIIa,

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