Ağır Hemofili A Hastasında Anestezi Yönetimi
Özet
Hemofili A kalıtsal bir kanama bozukluğudur. Kas ve eklem içi kanamalarla kendini gösterir ve faktör VIII eksikliği nedeniyle gelişir. X’e bağlı resesif geçiş gösteren hemofili A, yaklaşık 5.000-10.000 erkek doğumunda bir görülür (1). Hastalığın ağırlık derecesi arttıkça, tanı daha erken konmakta ve daha şiddetli klinik bulgular görülmektedir. Faktör VIII aktivitesi <%1 olan hastalar “ağır hemofili” kliniği gösterirken, %1-5 arasında olanlar “orta hemofili” ve >%5 olanlar “hafif hemofili” kliniği gösterirler (1).
İzole uzamış aktive parsiyel tromboplastin zamanı değeri faktör VIII eksikliği için uyarıcı olmalıdır. Tedavi faktör VIII’in yerine konmasıdır. (2). Bu olgu sunumunda laparoskopik üreterolitotomi cerrahisi geçiren ve faktör VIII eksikliği bulunan 43 yaşındaki erkek hastanın anestezi yönetimi sunulmaktadır.
Hemophilia A is an inherited bleeding disorder that develops as a result of factor VIII deficiency and manifests itself with intra-articular and intramuscular bleeding. Hemophilia A, which shows X-linked recessive inheritance, is observed in approximately 5.000-10.000 male births (1). As the severity of the disease increases, the diagnosis is made earlier and more severe clinical findings are observed. Patients with factor activity <1% show “severe hemophilia” clinic, while those with factor activity between 1-5% show “moderate hemophilia” and those with factor activity >5% show “mild hemophilia” clinic (1).
Isolated prolonged activated partial thromboplastin time should be a warning sign for factor VIII deficiency. Treatment is replacement of factor VIII. (2). In this case report, anesthesia management of a 43-year-old male patient with factor VIII deficiency who underwent laparoscopic ureterolithotomy surgery is presented.
Referanslar
Kar A, Phadnis S, Dharmarajan S et al. Epidemiology & social costs of haemophilia in India. Indian J Med Res. 2014;140:19–31.
Mallya P, Kaimar P, Jithesh R et al. Anaesthetic management of a patient with haemophilia. Indian J Anaesth. 2007;51:145.
Mistry T, Dogra N, Chauhan K et al. Perioperative Considerations in a Patient with Hemophilia A: A Case Report and Review of Literature. Anesth Essays Res. 2017;11(1):243-245. doi: 10.4103/0259-1162.181432.
Gyanesh P, Dhiraaj S. Anesthetic management of a patient with hemophilia A with spontaneous acute subdural hematoma. J Anaesthesiol Clin Pharmacol. 2013;29(1):117-20. doi: 10.4103/0970-9185.105819.
Flores RP, Bagatini A, dos Santos AT et al. Hemophilia and Anesthesia. Rev Bras Anestesiol. 2004; 54:865-7. doi: 10.1590/s0034-70942004000600017.
Ahmed HS, Jayaram PR. Anesthetic considerations for joint replacement surgery in hemophilic arthropathy. Anesth Pain Med (Seoul). 2024 Jul;19(3):194-208. doi: 10.17085/apm.24047.
Santagostino E, Escobar M, Ozelo M et al. Recombinant activated factor VII in the treatment of bleeds and for the prevention of surgery-related bleeding in congenital haemophilia with inhibitors. Blood Rev. 2015;29(1):9-18. doi: 10.1016/S0268-960X(15)30003-5.
Hay CR, Palmer B, Chalmers E et al. Incidence of factor VIII inhibitors throughout life in severe hemophilia A in the United Kingdom. Blood. 2011 Jun 9;117(23):6367-70. doi: 10.1182/blood-2010-09-308668. Epub 2011 Apr 6. PMID: 21471523.
Konkle BA, Ebbesen LS, Erhardtsen E et al. Randomized, prospective clinical trial of recombinant factor VIIa for secondary prophylaxis in hemophilia patients with inhibitors. J Thromb Haemost. 2007;5(9):1904-13.
Riley RR, Witkop M, Hellman E et al. Assessment and management of pain in haemophilia patients. Haemophilia. 2011;17:839–45. doi:10.1111/j.1365-2516.2011.02567.x
Jover-Cerveró A, Poveda Roda R, Bagán JV et al. Dental treatment of patients with coagulation factor alterations: An update. Med Oral Patol Oral Cir Bucal. 2007;12:E 380–7.
Thornburg CD, Duncan NA. Treatment adherence in hemophilia. Patient Prefer Adherence. 2017 Sep 27;11:1677-1686. doi: 10.2147/PPA.S139851.
Referanslar
Kar A, Phadnis S, Dharmarajan S et al. Epidemiology & social costs of haemophilia in India. Indian J Med Res. 2014;140:19–31.
Mallya P, Kaimar P, Jithesh R et al. Anaesthetic management of a patient with haemophilia. Indian J Anaesth. 2007;51:145.
Mistry T, Dogra N, Chauhan K et al. Perioperative Considerations in a Patient with Hemophilia A: A Case Report and Review of Literature. Anesth Essays Res. 2017;11(1):243-245. doi: 10.4103/0259-1162.181432.
Gyanesh P, Dhiraaj S. Anesthetic management of a patient with hemophilia A with spontaneous acute subdural hematoma. J Anaesthesiol Clin Pharmacol. 2013;29(1):117-20. doi: 10.4103/0970-9185.105819.
Flores RP, Bagatini A, dos Santos AT et al. Hemophilia and Anesthesia. Rev Bras Anestesiol. 2004; 54:865-7. doi: 10.1590/s0034-70942004000600017.
Ahmed HS, Jayaram PR. Anesthetic considerations for joint replacement surgery in hemophilic arthropathy. Anesth Pain Med (Seoul). 2024 Jul;19(3):194-208. doi: 10.17085/apm.24047.
Santagostino E, Escobar M, Ozelo M et al. Recombinant activated factor VII in the treatment of bleeds and for the prevention of surgery-related bleeding in congenital haemophilia with inhibitors. Blood Rev. 2015;29(1):9-18. doi: 10.1016/S0268-960X(15)30003-5.
Hay CR, Palmer B, Chalmers E et al. Incidence of factor VIII inhibitors throughout life in severe hemophilia A in the United Kingdom. Blood. 2011 Jun 9;117(23):6367-70. doi: 10.1182/blood-2010-09-308668. Epub 2011 Apr 6. PMID: 21471523.
Konkle BA, Ebbesen LS, Erhardtsen E et al. Randomized, prospective clinical trial of recombinant factor VIIa for secondary prophylaxis in hemophilia patients with inhibitors. J Thromb Haemost. 2007;5(9):1904-13.
Riley RR, Witkop M, Hellman E et al. Assessment and management of pain in haemophilia patients. Haemophilia. 2011;17:839–45. doi:10.1111/j.1365-2516.2011.02567.x
Jover-Cerveró A, Poveda Roda R, Bagán JV et al. Dental treatment of patients with coagulation factor alterations: An update. Med Oral Patol Oral Cir Bucal. 2007;12:E 380–7.
Thornburg CD, Duncan NA. Treatment adherence in hemophilia. Patient Prefer Adherence. 2017 Sep 27;11:1677-1686. doi: 10.2147/PPA.S139851.
