Kanama Diyatezi Olan Olguda Perioperatif Anestezi Yönetimi
Özet
Bu vaka sunumu, 59 yaşında, rahim sarkması nedeniyle vajinal histerektomi planlanan ve ameliyat öncesi tetkiklerinde nadir görülen bir pıhtılaşma bozukluğu saptanan bir hastanın perioperatif anestezi yönetimini ele almaktadır. Hastanın yapılan ileri tetkiklerinde Faktör VIII ve von Willebrand faktör (vWf) düzeylerinin ileri derecede düşük olduğu, ayrıca FVIII inhibitörünün pozitif olduğu saptanmıştır. Bu bulgular ışığında hastaya edinsel von Willebrand hastalığı ve edinsel hemofili A tanısı konulmuştur. Hematoloji bölümü, olası bir kanama durumunda standart faktör konsantreleri yerine "bypas" edici ajanların (aPCC ve rVIIa) kullanılmasını önermiştir. Ameliyat sırasında genel anestezi uygulanmış ve hemodinamik stabilite korunmuştur. Ameliyat sonrası derlenme ünitesinde gelişen vajinal kanama, yatak başı Rotasyonel Tromboelastometri (ROTEM) testi ile değerlendirilmiş ve analizlerin normal gelmesi üzerine kanama sadece 1 gram intravenöz traneksamik asit (TXA) ile kontrol altına alınmıştır. Hasta, postoperatif dördüncü günde sorunsuz bir şekilde taburcu edilmiştir. Sonuç olarak, bu tür karmaşık kanama bozukluklarında multidisipliner yaklaşım, doğru tanı ve ROTEM gibi viskoelastik testlerin kullanımı, gereksiz ve riskli faktör kullanımını önleyerek güvenli bir cerrahi süreç yönetimi sağlamaktadır.
This case report discusses the perioperative anesthesia management of a 59-year-old patient scheduled for vaginal hysterectomy due to uterine prolapse, in whom a rare bleeding disorder was detected during preoperative tests. Advanced evaluations revealed significantly low levels of Factor VIII and von Willebrand factor (vWf), along with positive FVIII inhibitors. Based on these findings, the patient was diagnosed with concurrent acquired von Willebrand disease and acquired hemophilia A. The hematology department recommended the use of "bypassing" agents (aPCC and rVIIa) instead of standard factor concentrates in the event of bleeding. General anesthesia was administered during surgery, and hemodynamic stability was maintained. Vaginal bleeding that occurred in the recovery unit postoperatively was evaluated using bedside Rotational Thromboelastometry (ROTEM); as the analyses were within normal limits, the bleeding was controlled with only 1 gram of intravenous tranexamic acid (TXA). The patient was discharged without complications on the fourth postoperative day. In conclusion, a multidisciplinary approach, accurate diagnosis, and the use of viscoelastic tests like ROTEM in such complex bleeding disorders ensure safe surgical management by preventing unnecessary and risky factor administration.
Referanslar
Rodeghiero, F. G. Castaman, and E. Dini, Epidemiological investigation of the prevalence of von Willebrand’s disease. Blood 1987 Feb;69(2):454-9.
Sadler JE, Mannucci PM, Berntorp E, Bochkov N, Boulyjenkov V, Ginsburg D et al. Impact, diagnosis and treatment of von Willebrand disease. Thromb Haemost 2000 Aug;84(2):160-74
Dr. Mindy L. Simpson, HematoLog 2012: 2 ■ 2
Simone JV, Cornet JA, Abildgaard CF. Acquired von Willebrand’s syndrome in systemic lupus erythematosus. Blood 1968; 31:806–812
Vincentelli A, Susen S, Le Tourneau T, et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med 2003; 349:343–349.
Stone ME, Mazzeffi M, Derham J, Korshin A. Current management of von Willebrand disease and von Willebrand syndrome. Curr Opin Anaesthesiol 2014 Jun;27(3):353-8.
Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M et al; UK Haemophilia Centre Doctors' Organisation. Acquired haemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation. Blood 2007;109:1870-1877.
Franchini M. Postpartum Acquired Factor VIII Inhibitors. Am J Hematol 2006;81:768-773.
Hsieh C-C, Chen H-H, Chen D-Y, Lan J-L. Systemic lupus erythematosus develop seven years after acquisition of acquired haemophilia A in a patient with palindromic rheumatism: a case report. Formosan Journal of Rheumatology 2009;23:47-51.
Kyaw TZ, Jayaranee S, Bee PC, Chin EF. Acquired Factor VIII Inhibitors: Three Cases. Turk J Haematol 2013 Mar;30(1):76-80.
O'Donnell JS, Lavin M. Perioperative management of patients with von Willebrand disease. Hematology Am Soc Hematol Educ Program 2019 Dec 6;2019(1):604-609.
Miesbach W. Perioperative management for patients with von Willebrand disease: Defining the optimal approach. Eur J Haematol 2020 Oct;105(4):365-377.
Franchini M, Gandini G, Paolantonio TD, Mariani G. Acquired Haemophilia A: A concise review. Am J Hematol 2005;80:55-63.
Kaufmann JE, Vischer UM. Cellular mechanisms of the hemostatic effects of desmopressin (DDAVP). J Thromb Haemost 2003 Apr;1(4):682-9.
Windyga J, Dolan G, Altisent C, Katsarou O, L´opez Fern´andez MF, Z¨ulfikar B; EHTSB. Practical aspects of factor concentrate use in patients with von Willebrand disease undergoing invasive procedures: a European survey. Haemophilia 2016;22(5):739-751.
Roberts I, Shakur H, Coats T, et al. The CRASH-2 trial: a randomised controlled trial and economic evaluation of the effects of tranexamic acid on death, vascular occlusive events and transfusion requirement in bleeding trauma patients. Health Technol Assess 2013;17(10):1-79.
WOMAN Trial Collaborators. Effect of early tranexamic acid administration on mortality, hysterectomy, and other morbidities in womenwith post-partum haemorrhage (WOMAN): an international, randomised, double-blind, placebo-controlled trial. Lancet 2017;389(10084): 2105-2116.
Huth-Kuhne A, Baudo F, Collins P, Ingerslev J, Kessler CM, Levesque H. International recommendations on the diagnosis and treatment of patients with acquired haemophilia A. Haematologica 2009;94:566-573.
Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, Villar A. Acquired Haemophilia: review and meta-analysis focused on therapy and prognostic factors. Br J Haematol 2003;121:21-35.
Franchini M, Lippi G. Acquired factor VIII inhibitors. Blood 2008;112:250-255.
Lottenberg R, Kentro TB, Kitchens CS. Acquired haemophilia A. A natural history study of 16 patients with factor VIII inhibitors receiving little or no therapy. Arch Intern Med 1987;147:1077-1081.
Green D, Rademaker AW, Briët E. A prospective, randomized trial of prednisone and cyclophosphamide in the treatment of patients with factor VIII autoantibodies. Thromb Haemost 1993;70:753-757.
Pareti FI, Lattuada A, Bressi C, et al. Proteolysis of von Willebrand factor and shear stress-induced platelet aggregation in patients with aortic valve stenosis. Circulation 2000; 102:1290–1295.
Milaskiewicz RM, Holdcroft A, Letsky E. Epidural anaesthesia and von Willebrand's disease. Anaesthesia 1990 Jun;45(6):462-4.
Marrache D, Mercier FJ, Boyer-Neumann C, Roger-Christoph S, Benhamou D. Epidural analgesia for parturients with type 1 von Willebrand disease. Int J Obstet Anesth 2007 Jul;16(3):231-5.
Butwick AJ, Carvalho B. Neuraxial anesthesia for cesarean delivery in a parturient with type 1 von Willebrand disease and scoliosis. J Clin Anesth 2007 May;19(3):230-3.
Jones BP, Bell EA, Maroof M. Epidural labor analgesia in a parturient with von Willebrand's disease type IIA and severe preeclampsia. Anesthesiology 1999 Apr;90(4):1219-20.
Hepner DL, Tsen LC. Severe thrombocytopenia, type 2B von Willebrand disease and pregnancy. Anesthesiology 2004 Dec;101(6):1465-7.
Laffan MA, Lester W, O’Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol 2014;167(4): 453-465.
Sørensen B, Ingerslev J. Tailoring haemostatic treatment to patient requirements - an update on monitoring haemostatic response using thrombelastography. Haemophilia 2005 Nov;11 Suppl 1:1-6.
Tran HTT, Sørensen B, Bjørnsen S, Pripp AH, Tjønnfjord GE, Andre Holme P. Monitoring bypassing agent therapy - a prospective crossover study comparing thromboelastometry and thrombin generation assay. Haemophilia 2015 Mar;21(2):275-283.
Schmidt DE, Majeed A, Bruzelius M, Odeberg J, Holmström M, Ågren A. A prospective diagnostic accuracy study evaluating rotational thromboelastometry and thromboelastography in 100 patients with von Willebrand disease. Haemophilia 2017 Mar;23(2):309-318.
Topf HG, Strasser ER, Breuer G, Rascher W, Rauh M, Fahlbusch FB. Closing the gap - detection of clinically relevant von Willebrand disease in emergency settings through an improved algorithm based on rotational Thromboelastometry. BMC Anesthesiol 2019 Jan 10;19(1):10.
Curry NS, Davenport R, Pavord S, Mallett SV, Kitchen D, Klein AA et al. The use of viscoelastic haemostatic assays in the management of major bleeding: A British Society for Haematology Guideline. Br J Haematol 2018 Sep;182(6):789-806.
Hans GA, Besser MW. The place of viscoelastic testing in clinical practice. Br J Haematol 2016 Apr;173(1):37-48.
Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008;14(2):171-232.
