Kalıtsal Hemoglobinopatilerde Zor Karar: Kan Transfüzyonu; Ne Zaman, Nasıl, Niçin?

Orak hücreli anemi ve talasemi, dünyada en sık görülen kalıtsal hemoglobinopatilerdir. Kronik hemolitik anemi ile seyrederler. Yaşam kaliteleri için aralıklı kan transfüzyonuna gereksinim duyarlar. Sıklıkla dokuya oksijen sunumunda sorun yaşayan bu hastalarda erken yaşlarda çoklu organ yetmezlikleri gelişir. Kan transfüzyonunun yan etkilerini sık yaşayan ve organ fonksiyonları sınırda olan bu hastaların anestezi yönetimleri oldukça zor ve karmaşıktır. Ne yazık ki bugüne kadar hastalarda perioperatif komplikasyonların azaltılabilmesi açısından gerekli transfüzyon miktarı ve hemoglobin düzeyleri konusunda, kanıta dayalı, ortak bir görüş oluşturulamamıştır. Literatürde varolan veriler, özellikle preoperatif döneme odaklanmış, az sayıdaki randomize kontrollü çalışmalar ve olgu sunumlarıdır. Bu derlemede orak hücreli anemi ve talasemiler ayrı başlıklar halinde ele alınmış, hemoglobinopatili hastalara uygulanan perioperatif transfüzyonların gereklilikleri değerlendirilmeye çalışılmıştır.

Difficult Decision in Hereditary Hemoglobinopathies: Blood transfusion; when, how and why?

Sickle cell anemia and thalassaemia are the most common hereditary hemoglobinopathies in the world. They course with chronic hemolytic anemia. These patients require intermittent blood transfusion for their quality of life. Multiple organ failure develops at an early age in these patients, who frequently experience problems in delivering oxygen to the tissue. The anesthetic management of these patients who have frequent side effects of blood transfusion and whose organ functions are at the limit is quite difficult and complicated. Unfortunately, no evidencebased, commonly shared opinion has been established about the amount of transfusion required and desired hemoglobin levels to reduce perioperative complications in these patients. The data available in the literature are derived from scarce number of randomized controlled trials and case reports, especially focused on the preoperative period. In this review, sickle cell anemia and thalassemia are treated as separate headings and the indications of perioperative transfusions applied to patients with hemoglobinopathy have been evaluated.

___

  • Malik P. Gene Therapy for hemoglobinopathies: Tremendous successes and remaining caveats. Mol Ther. 2016;24:668-70. https://doi.org/10.1038/mt.2016.57
  • Khurmi N, Gorlin A, Misra L. Perioperative considerations for patients with sickle cell disease: a narrative review. Can J Anaesth. 2017; 64:860-9. https://doi.org/10.1007/s12630-017-0883-3
  • Modell B, Darlison MW, Moorthie S, Blencowe H, Petrou M, Lawn J. Epidemiologic methods in community genetics and model global database of congenital disorders, 2016.
  • Tsaras G, Owusu-Ansah A, Boateng FO, AmoatengAdjepong Y. Complications associated with sickle cell trait: a brief narrative review. Am J Med. 2009;122:507-12. https://doi.org/10.1016/j.amjmed.2008.12.020
  • Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions. PLoS Med. 2013;10:e1001484. https://doi.org/10.1371/journal.pmed.1001484
  • Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med. 2011;41:398-405. https://doi.org/10.1016/j.amepre.2011.09.013
  • Lovaglio PG. Patient safety analysis linking claims and administrative data. Int J Health Care Qual Assur. 2012;25:698-711. https://doi.org/10.1108/09526861211270640
  • Adjepong KO, Otegbeye F, Adjepong YA. Perioperative management of sickle cell disease. Mediterr J Hematol Infect Dis. 2018;10:e2018032. https://doi.org/10.4084/mjhid.2018.032
  • Higgs DR, Engel JD, Stamatoyannopoulos G. Thalassaemia. Lancet. 2012;379:373-83. https://doi.org/10.1016/S0140-6736(11)60283-3
  • Piga A, Serra M, Longo F, et al. Changing patterns of splenectomy in transfusion-dependent thalassemia patients. Am J Hematol. 2011;86:808-10. https://doi.org/10.1002/ajh.22102
  • Ashley-Koch A, Yang Q, Olney RS. Sickle hemoglobin (Hb S) allele and sickle cell disease: a HuGE review. Am J Epidemiol. 2000;151:839-45. https://doi.org/10.1093/oxfordjournals.aje.a010288
  • National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: expert panel report. Washington, DC: National Institutes of Health, 2014.
  • McCurdy PR, Sherman AS. Irreversibly sickled cells and red cell survival in sickle cell anemia: a study with both DF32P and 51CR. Am J Med. 1978;64:253-8. https://doi.org/10.1016/0002-9343(78)90053-0
  • Eadie GS, Brown IW. Analytical review: red blood cell survival studies. Blood. 1953;8:1110-36.
  • Reiter CD, Wang X, Tanus-Santos JE, et al. Cell-free hemoglobin limits nitric oxide bioavailability in sicklecell disease. Nat Med. 2002;8:1383-9. https://doi.org/10.1038/nm1202-799
  • Lancaster JR. Reaping of nitric oxide by sickle cell disease. Proc Natl Acad Sci. 2002;99:552-3. https://doi.org/10.1073/pnas.032633399
  • Robinson JM, Lancaster JR Jr. Hemoglobin-mediated, hypoxia-induced vasodilation via nitric oxide: mechanism(s) and physiologic versus pathophysiologic relevance. Am J Respir Cell Mol Biol. 2005;32:257-61. https://doi.org/10.1165/rcmb.F292
  • Piel FB, Patil AP, Howes RE, et al. Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun. 2010;2:104. https://doi.org/10.1038/ncomms1104
  • Schnog JB, Duits AJ, Muskiet FA, ten Cate H, Rojer RA, Brandjes DP. Sickle cell disease; a general overview. Neth J Med. 2004;62:364-74.
  • Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;86:480-7. https://doi.org/10.2471/BLT.06.036673
  • Arcasoy A. Dünya’da ve Türkiye’de talasemi ve hemoglobinopatiler. Hemoglobinopati ve Talasemi ÖnlemTanı-Tedavi Ulusal Hemoglobinopati Konseyi Sağlık Bakanlığı. 2003;2:11-9, Antalya.
  • Canatan D, Aydınok Y. Talasemi ve Hemoglobinopatiler. Tanı ve Tedavi; Retma Matbaacılık Antalya, 2007; 11-19.
  • Kılınç Y, Akmanlar N, Kümi M, Köker I. The incidences of hemoglobinopathies and thalassemias in cord blood of newborns from Çukurova Province. Med Bull. 1992;25:9-14.
  • Al-Mulhim AS, Alshehri MH. Laparoscopic cholecystectomy in adult patients with sickle cell disease. Surg Laparosc Endosc Percutan Tech. 2012;22:454-8. https://doi.org/10.1097/SLE.0b013e3182619408
  • Delatte SJ, Hebra A, Tagge EP, et al. Acute chest syndrome in the postoperative sickle cell patient. J Pediatr Surg. 1999;34:188-92. https://doi.org/10.1016/S0022-3468(99)90254-3
  • Howard J. Sickle cell disease: when and how to transfuse. Am Soc Hematol Educ Prog. 2016;2:625-31. https://doi.org/10.1182/asheducation-2016.1.625
  • Kanter J, Kruse-Jarres R. Management of sickle cell disease from childhood through adulthood. Blood Rev. 2013;27:279-87. https://doi.org/10.1016/j.blre.2013.09.001
  • Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med. 1995;332:1317-22. https://doi.org/10.1056/NEJM199505183322001
  • Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014;312:1033-48. https://doi.org/10.1001/jama.2014.10517
  • Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med. 1995;333:206-13. https://doi.org/10.1056/NEJM199507273330402
  • Chou ST. Transfusion therapy for sickle cell disease: a balancing act. Am Soc Hematol Educ Prog. 2013;2013:439-46. https://doi.org/10.1182/asheducation-2013.1.439
  • Alotaibi GS, Alsaleh K, Bolster L, Sean McMurtry M, Wu C. Preoperative transfusion in patients with sickle cell disease to prevent perioperative complications: A systematic review and meta-analysis. Hematology. 2014;19:463-71. https://doi.org/10.1179/1607845414Y.0000000158
  • Koshy M, Weiner SJ, Miller ST, Sleeper LA, Vichinsky E, Brown AK, et al. Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases. Blood. 1995;86:3676-84.
  • Howard J, Malfroy M, Llewelyn C, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. The Lancet. 2013;381:930-8. https://doi.org/10.1016/S0140-6736(12)61726-7
  • Al-Jaouni S, Al-Muhayawi S, Qari M, Nawas MA, AbdelRazeq H. The safety of avoiding transfusion preoperatively in patients with sickle cell hemoglobinopathies. Blood. 2002;100:1-9.
  • Edwin F, Aniteye E, Tettey M, et al. Hypothermic cardiopulmonary bypass without exchange transfusion in sickle-cell patients: a matched-pair analysis. Interact Cardiovasc Thorac Surg. 2014;19:771-6. https://doi.org/10.1093/icvts/ivu249
  • Roizen MF. Anesthesia implications of concurrent diseases. In: Miller RD, ed. Anesthesia. 5th ed. Philadelphia: Chuchill Livingstone, 2000.
  • Fisher B, Roberts CS. Tourniquet use and sickle cell hemoglobinopathy: how should we proceed?. South Med J. 2010;103:1156-60. https://doi.org/10.1097/SMJ.0b013e3181efaf3b
  • Giardine B, Borg J, Higgs DR, et al. Systematic documentation and analysis of human genetic variation in hemoglobinopathies using the microattribution approach. Nat Genet. 2011;43:295-301. https://doi.org/10.1038/ng.785
  • Aessopos A, Farmakis D, Karagiorga M, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood. 2001;97:3411-6. https://doi.org/10.1182/blood.V97.11.3411
  • C. Staikou, E. Stavroulakis, Karmaniolou I. A narrative review of peri-operative management of patients with thalassaemia. Anaesthesia. 2014;69:494-510. https://doi.org/10.1111/anae.12591
  • Firth PG. Anesthesia and hemoglobinopathies. Anesthesiol Clin. 2009;27:321-36. https://doi.org/10.1016/j.anclin.2009.05.001
  • Akar N, Çavdar AO, Dessi E, Loi A, Pirastu M, Cao A. β-thalassemia mutations in the Turkish population. J Med Genet. 1987;24:378-9. https://doi.org/10.1136/jmg.24.6.378
  • Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, et al. Survival and complications in thalassemia. Ann N Y Acad Sci. 2005;1054:40-7. https://doi.org/10.1196/annals.1345.006
  • Abi Saad GS, Musallam KM, Taher AT. The surgeon and the patient with b-thalassaemia intermedia. Br J Surg. 2011;98:751-60. https://doi.org/10.1002/bjs.7533
  • Orr D. Difficult intubation: a hazard in thalassaemia. A case report. Br J Anaesth. 1967;39:585-6. https://doi.org/10.1093/bja/39.7.585
  • Ali S, Khan FA. Anaesthetic management of two patients with beta-thalassaemia intermedia. J Pak Med Assoc. 2010;60:582-4.
  • Voyagis GS, Kyriakis KP. Homozygous thalassemia and difficult endotracheal intubation. Am J Hematol. 1996;52:125-6. https://doi.org/10.1002/(SICI)1096- 8652(199606)52:2<125::AID-AJH15>3.0.CO;2-G
  • Perez JA, Padilla J, Rodriguez MA, Cura S, et al. Splenectomy in a patient with beta thalassemia intermedia and severe hemolytic anemia. Rev Esp Anestesiol Reanim. 2001;48:288-91.
  • Suwanchinda V, Tengapiruk Y, Udomphunthurak S. Hypertension perioperative splenectomy in thalassemic children. J Med Assoc Thai. 1994;77:66-70.
  • Taher A, Isma’eel H, Mehio G, et al. Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran. Thromb Haemost. 2006;96:488-91. https://doi.org/10.1160/TH06-05-0267
  • Butwick A, Findley I, Wonke B. Management of pregnancy in a patient with b thalassaemia major. Int J Obstet Anesth. 2005;14:351-4. https://doi.org/10.1016/j.ijoa.2005.02.002
  • Katz R, Goldfarb A, Muggia M, Gimmon Z. Unique features of laparoscopic cholecystectomy in beta thalassemia patients. Surg Laparosc Endosc Percutan Tech. 2003;13:318-21. https://doi.org/10.1097/00129689-200310000-00006
  • Perez Ferrer A, Ferrazza V, Gredilla E, Buendia JV, Rua A, Larrea A. Bloodless surgery in a patient with thalassemia minor. Usefulness of erythropoietin, preoperative blood donation and intraoperative blood salvage. Minerva Anestesiol. 2007;73:323-6.
  • Waters JH, Lukauskiene E, Anderson ME. Intraoperative blood salvage during cesarean delivery in a patient with b thalassemia intermedia. Anesth Analg. 2003;97:1808-09. https://doi.org/10.1213/01.ANE.0000087046.91072.E8
  • Suwanchinda V, Tanphaichitr V, Pirayavaraporn S, Somprakit P, Laohapensang M. Hemodynamic responses to captopril during splenectomy in thalassemic children. J Med Assoc Thai. 1999;82:666-71.
Anestezi Dergisi-Cover
  • ISSN: 1300-0578
  • Yayın Aralığı: Yılda 4 Sayı
  • Başlangıç: 1993
  • Yayıncı: Betül Kartal
Sayıdaki Diğer Makaleler

Geriyatrik Hastaların Dental Tedavilerinde Anestezi Uygulamalarımız

HATİCE AKPINAR

Airway Management Experiences In Bariatric Surgery

Betül KOCAMER ŞİMŞEK, Yunus BAYDİLEK

Laparoskopik Kolesistektomide Pozitif Basınçlı Ventilasyon Sırasında Baska Mask, Proseal LMA ve I Gel ile Prospektif Randomize Karşılaştırmalı Bir Çalışma

Ramkumar DHANASEKARAN, Gautam Dilip MEHTA, Aruna PARAMESWARI

Spinal Anestezi ile Sezaryen Yapılacak Gebelerde Pasif Bacak Kaldırma Uygulamasının Hipotansiyonu Önlemedeki Etkinliği

Mehmet Emin İNCE, Ali SIZLAN, SERKAN ŞENKAL, Tarık PURTULOĞLU, Umut KARA, GÖKHAN ÖZKAN, Ercan KURT

Çocuk Hastalarda Fleksibl Fiberoptik Bronkoskopide Laringeal Maske Uygulaması: 125 Olgunun Değerlendirilmesi

Mine AKIN, Feyza SEVER, Sibel SAYDAM, Sengül ÖZMERT, Yeşim ŞENAYLI, Devrim Tanıl KURT, Gülsen KESKİN, Güzin CİNEL

Modifiye Radikal Mastektomi ve Aksiller Lenf Nodu Diseksiyonunda Ultrason Eşliğinde Yapılan Yüzeyel Serratus Plan Bloğunun Akut Postmastektomi Ağrısına Etkisi: Randomize Kontrollü Çalışma

Ahmet Murat YAYIK, Ali AHISKALIOĞLU, Muhammet Mustafa SULAK, Elif Oral AHISKALIOĞLU, Muhammet AHMET KARAKAYA, Erkan Cem ÇELİK, Erdem KARADENİZ, Hacı Ahmet ALICI

Epidural Steroid Enjeksiyonu Sonrasında Gelişen Bir Herpes Zoster Olgusu

Bülent Barış GÜVEN, Fulya YURTSEVER, Temel GÜNER, Sedat TEMİRCAN, Ömer BAKAL, AYŞIN ERSOY

Comparison of 3 Different Bupivacaine Concentrations Used in the Ultrasound Guided Infraclavicular Brachial Plexus Block

SEMİH BAŞKAN, Fahri ACAR, Gökhan DEMİRELLİ, Hidayet ÜNAL

Pediyatrik Alt Ekstremite Cerrahisi için Ultrason Eşliğinde Distal Adduktor Kanal Bloğu

Ali AHISKALIOĞLU, Ahmet Murat YAYIK, Erkan Cem ÇELİK

Laryngeal Mask Airway Application During Flexible Fiberoptic Bronchoscopy in Pediatric Patients: Evaluation of 125 Cases

Gülşen KESKİN, Mine AKIN, Yeşim ŞENAYLI, Sibel SAYDAM, Devrim Tanıl KURT, Sengül ÖZMERT, Feyza SEVER, Güzin CİNEL