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.