Glanzmann Trombastenisi Olan Septoplasti Hastasında Anestezi Yönetimi
Özet
On yedi yaşında 57 kg olan erkek hasta acil servis kliniğine epistaksis şikayetiyle başvurdu. Acil serviste ilk değerlendirilmesi yapılan hastanın anamnezinde altı yaşında fimozis açılması ve sünnet ameliyatı yapıldıktan sonra üç gün sızıntı tarzında kanaması olduğu için pediatrik cerrah tarafından çocuk hematoloji konsültasyonu istendiği ve yapılan tetkikler sonrası Glanzmann Trombastenisi (GT) tanısı konulduğu öğrenildi. Bu şikayetlerinin traneksamik asit ve trombosit süspansiyonu infüzyonu sonrasında düzeldiği öğrenildi. Ayrıca olgunun anamnezinde, birkaç kez diş eti kanaması geçirdiği ancak herhangi bir tedavi uygulamadan kanamanın kendiliğinden durduğu tespit edildi. Epistaksisi olan hasta için Kulak Burun Boğaz (KBB) kliniğinden konsültasyon istendi. Olgunun burun anterior ve posterioruna KBB konsültan hekimi tarafından nasal tamponlar yerleştirildi. KBB tarafından septoplasti cerrahisi planlandı. GT tanısı olan hastaya ayrıca hematoloji konsültasyonu istendi. Rutin kan tetkiklerinde ölçülen hematolojik laboratuvar değerleri; trombosit:172.000 µL⁻¹,Hb:10.9 gdL⁻¹, APTT:25.4 saniye, INR:1.11 ölçüldü. Periferik yaymada trombositlerde agregasyon defekti olduğu ve tek tek dağıldığı, eritrositlerde ise hipokromi olduğu tespit edildi. Hematolog tarafından ayrıca daha önce yapılan tetkikleri incelendi ve flow sitometride CD41 ve CD61 eksikliği tanımlandığı tespit edildi. Hematoloji ve KBB klinikleri tarafından beraber değerlendirilen hastanın on beş gün sonra gerekli kan ve kan ürünleri önceden hazırlanarak septoplasti ameliyatı olmasına karar verildi.
A 17-year-old male patient weighing 57 kg applied to the emergency department with epistaxis. It was learned that the patient, who was first evaluated in the emergency department, had a history of phimosis at the age of six and circumcision surgery and had three days of leakage bleeding, so a pediatric surgeon requested a pediatric hematology consultation and diagnosed him with Glanzmann Thrombasthenia (GT) after the examinations. It was learned that these complaints improved after tranexamic acid and platelet suspension infusion. In addition, it was determined in the patient's history that he had experienced gum bleeding several times, but the bleeding stopped on its own without any treatment. Consultation was requested from the Ear, Nose and Throat (ENT) clinic for the patient with epistaxis. Nasal tamponades were placed anteriorly and posteriorly by the ENT consultant physician. Septoplasty surgery was planned by the ENT. The patient diagnosed with GT was also asked for a hematology consultation. Hematology laboratory values measured in routine blood tests were as follows; platelet: 172,000 µL⁻¹,Hb: 10.9 g dL⁻¹,APTT: 25.4 seconds, INR: 1.11. In the peripheral smear, it was determined that there was an aggregation defect in the platelets and that they were dispersed individually, and there was hypochromia in the erythrocytes. The hematologist also reviewed the previous tests and determined that CD41 and CD61 deficiency was identified in the flow cytometry. The platelet aggregation test agglutination rates were; adenosine diphosphate 24%, adrenaline 15%, collagen 36% and ristocetin 95% and were compatible with GT. The patient was evaluated by the hematology and ENT clinics together and it was decided to have septoplasty surgery fifteen days later by preparing the necessary blood and blood products in advance.
Referanslar
Caen J, Cousin C. ["In vivo" disorder of platelet adhesiveness in Willebrand's disease and Glanzmann's thrombasthenias. Trial interpretation]. Nouv Rev Fr Hematol. 1962;2:685-694.
Nurden AT, Caen JP. An abnormal platelet glycoprotein pattern in three cases of Glanzmann's thrombasthenia. Br J Haematol. 1974;28(2):253-260.
Phillips DR, Agin PP. Platelet membrane defects in Glanzmann's thrombasthenia. Evidence for decreased amounts of two major glycoproteins. J Clin Invest. 1977;60(3):535-545.
Bennett JS. Hereditary disorders of platelet function. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al. (Eds). Hoffman Hematology: Basic Principles and Practice. 4th ed. Philadelphia: Churchill Livingstone Elsevier; 2005. p. 2327- 46.
Nurden AT, George JN. Inherited abnormalities of the platelet. In: Colman RW, Marder VJ, Clowes AW, George JN, Goldhaber S (Eds). Hemostasis and Thrombosis, 5th ed. Philadelphia: Lippincott Williams Wilkins; 2006. p. 987- 1010.
Poon MC, D'Oiron R, Von Depka M, et al.; International Data Collection on Recombinant Factor VIIa and Congenital Platelet Disorders Study Group. Prophylactic and therapeutic recombinant factor VIIa administration to patients with Glanzmann's thrombasthenia: results of an international survey. J Thromb Haemost 2004;2:1096-103.
French DL, Coller BS. Hematologically important mutations: Glanzmann trombasthenia. Blood Cells Mol Dis 1997;23:39-51.
Gropper M.A. Miller Anesthesia. (Ali Fuat Erdem Çev. Ed.). 9.Baskı. Ankara: Güneş Kitabevi; 2023.
Morgan GE, Mikhail MS, Murray MJ (editors). Sıvı Dengesi ve Transfüzyon. Klinik Anesteziyoloji. Tulunay M, Cuhruk H (editörler). 4. Baskı, Ankara: Güneş Kitabevi; 2008; 690-708.
Kutlubay B.,Özdemir GN., Tüysüz G.,ve ark. Glanzmann trombastenisi: Cerrahpaşa Tıp Fakültesi deneyimi. Turk Arch Ped 2012; 47:104-6.
Valentino LA. Use of rFVIIa in 4 children with Glanzmann thrombasthenia. J Pediatr Hematol Oncol 2006;28:653-8.
Wertz D, Boveroux P, Péters P, et al.Surgical resection of a sphenoid wing meningioma in a patient with Glanzmann thrombasthenia.Acta Anaesthesiol Belg 2011;62:83-6.
Referanslar
Caen J, Cousin C. ["In vivo" disorder of platelet adhesiveness in Willebrand's disease and Glanzmann's thrombasthenias. Trial interpretation]. Nouv Rev Fr Hematol. 1962;2:685-694.
Nurden AT, Caen JP. An abnormal platelet glycoprotein pattern in three cases of Glanzmann's thrombasthenia. Br J Haematol. 1974;28(2):253-260.
Phillips DR, Agin PP. Platelet membrane defects in Glanzmann's thrombasthenia. Evidence for decreased amounts of two major glycoproteins. J Clin Invest. 1977;60(3):535-545.
Bennett JS. Hereditary disorders of platelet function. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al. (Eds). Hoffman Hematology: Basic Principles and Practice. 4th ed. Philadelphia: Churchill Livingstone Elsevier; 2005. p. 2327- 46.
Nurden AT, George JN. Inherited abnormalities of the platelet. In: Colman RW, Marder VJ, Clowes AW, George JN, Goldhaber S (Eds). Hemostasis and Thrombosis, 5th ed. Philadelphia: Lippincott Williams Wilkins; 2006. p. 987- 1010.
Poon MC, D'Oiron R, Von Depka M, et al.; International Data Collection on Recombinant Factor VIIa and Congenital Platelet Disorders Study Group. Prophylactic and therapeutic recombinant factor VIIa administration to patients with Glanzmann's thrombasthenia: results of an international survey. J Thromb Haemost 2004;2:1096-103.
French DL, Coller BS. Hematologically important mutations: Glanzmann trombasthenia. Blood Cells Mol Dis 1997;23:39-51.
Gropper M.A. Miller Anesthesia. (Ali Fuat Erdem Çev. Ed.). 9.Baskı. Ankara: Güneş Kitabevi; 2023.
Morgan GE, Mikhail MS, Murray MJ (editors). Sıvı Dengesi ve Transfüzyon. Klinik Anesteziyoloji. Tulunay M, Cuhruk H (editörler). 4. Baskı, Ankara: Güneş Kitabevi; 2008; 690-708.
Kutlubay B.,Özdemir GN., Tüysüz G.,ve ark. Glanzmann trombastenisi: Cerrahpaşa Tıp Fakültesi deneyimi. Turk Arch Ped 2012; 47:104-6.
Valentino LA. Use of rFVIIa in 4 children with Glanzmann thrombasthenia. J Pediatr Hematol Oncol 2006;28:653-8.
Wertz D, Boveroux P, Péters P, et al.Surgical resection of a sphenoid wing meningioma in a patient with Glanzmann thrombasthenia.Acta Anaesthesiol Belg 2011;62:83-6.
