Anestesia dan Thalasemia - Saeful Alam, M. Deny; Sudjud, Reza Widianto; , Indriasari (Majalah Anestesia dan Critical Care, 2014)
Thalasemia merupakan penyakit keturunan atau herediter menurut hukum mandel yang melibatkan penurunan produksi salah satu atau lebih rantai globin (α,β,γ,δ) dari hemoglobin sehingga terjadi gangguan sintesis hemoglobin. Gejala sudah mulai terdeteksi sejak bulan pertama kehidupan ketika level Hb fetal menurun. Gejala klinis yang dijumpai biasanya berhubungan dengan anemia yang berat, erytropoisis yang inefektif, extramedular hematopoiesis, dan gejala yang muncul karena timbunnan tranfusion dan akibat peningkatan penyerapan besi. Kulit biasanya tampak pucat karena anemia dan kuning karena jaundice dari hiperbilirubinemia. Tulang kepala dan tulang-tulang yang lainnya biasanya mengalami deformitas karena erytroid hyperplasia dengan intramedullary expansion dan penipisan tulang kortek dikenal dengan facies colley. Pasien dengan thalasemia baik intermediate atau mayor pada suatu waktu mungkin memerlukan penanganan bedah seperti misalnya cholecystectomy ataupun spleenectomi sehingga memerlukan tindakan anestesi. Permasalahan yang perlu diperhatikan saat melakukan anestesi pasien thalasemia diantaranya komplikasi akibat anemia, komplikasi akibat timbunnan besi, dan komplikasi karena terapi chelation. Kata kunci: Anemia, anestesi, hemosiderosis, splenektomi, thalasemia Anaesthesia and Thalassaemia Thalassaemia is a hereditary disorder according to Mandel’s law, involving a reduction in one of the globin chains (α,β,γ,δ) from haemoglobin resulting in impaiment of haemoglobin synthesis. Sysmptoms may present as early as one month of life when there is reduction in fetal haemoglobin. Clinical symptoms usually relates to severe anemia, ineffective erythropoiesis and symptoms that occurs as a result of transfussion and iron loading. Skin usually appears pale due to anemia and yellow due to jaundice and hyperbilirubinemia. Deformity of skull and other bones usually occurs as a result of erythroid hyperplasia with intramedually expansion and thinning of cortex known as Facies Colley. Patients with thalassaemia, either intermediate or major, may require surgery some time in their life, such as cholecystectomy or splenectomy therefore requiring anaesthesia. Issues that need to be adressed during anaesthesia include complications due to anemia, iron loading and chelation therapy. Key words: Anemia, anaesthesia, haemosiderosis, splenectomy, thalassaemia Reference Lanzkowsky P. Manual of pediatric hematology and oncology. Edisi ke-4. Burlington: Elsevier Academic Press; 2005. Yaish HM. Thalasemia [diunduh 2 Januari 2011]. Tersedia dari: http//www.emedicine. com. DeBaun MR, Vichinsky E. Hemoglobinopathies. Dalam Kliegman RM, Behrman RE, Jenson HB, Stanton BF, penyunting. Nelson Textbook of Pediatrics. Edisi ke⎯18. Philadelphia: Elsevier Saunders; 2007. h. 2025⎯38. Permono B, Ugrasena IDG. Hemoglobin abnormal: talasemia. Dalam Permono B, Sutaryo, Ugrasena IDG, Windiastuti E, Abdulsalam M, penyunting. Buku ajar hematologi-onkologi anak. Cetakan ke-2. Jakarta: Badan Penerbit IDAI; 2006. hlm. 64-97. Lekawanvijt S, Chattipakorn N. Iron overload thalassemic cardiomyopathy: iron status assessment and mechanisms of mechanical and electrical disturbance due to iron toxicity. Can J Cardiol. 2009;25(4):213⎯8. Kushner JP, Porter JP, Olivieri NF. Secondary iron overload. Hematology. 2001:47⎯61. Rund D, Rachmilewitz E. β-Thalasemia. N Engl J Med. 2005;353:1135-46. Olivieri NF. The β-Thalasemia. N Engl J Med. 1999:341(2):99-109. Catlin AJ. Thalasemia: the facts and the controversies. Pediatr Nursing. 2003;29(6):447⎯51. Bahador A, Banani SA, Foroutan HR, Hosseini SM, Davani SZ. A comparative study of partial vs total splenectomy in thalasemia major patients. J Indian Assoc Pediatr Surg. 2007 vol 12, page 133⎯5 Grosfeld JL, dkk. The Spleen in Pediatric Surgery edisi ke 2 dan 6. Mosby Elsevier. 2006 hlm. 1691-1702. Wood JC. Cardiac complications in thalasemia major. PMC. 2010;33:81⎯6. Colan SD. Cardiomyopathies. Dalam: Keane JF, Lock JE, Fyler D. Nadas’ pediatric cardiology. Edisi ke⎯2. Philadelphia: Saunders Elsevier; 2006. hlm. 415⎯58. Cogliandro T, Derchi G, Mancuso L, Mayer MC, Pannone B, Pepe A. Guideline recommendations for heart complications in talasemia major. Cardiology Medi. 2008;9:515⎯25. Yao Fun Sun F, Anesthesiology Problem-Oriented Patient Management. Edisi ke 6. Lippincott Williams & Wilkins. 2008:986–987. Hines RL, Marschall KE. Stoelting’s Anesthesia and Co-Existing Disease. Edisi ke 5. Saunders Elsevier. 2008:412. Barash PG, Cullen BF, Stoelting RK, Calahan MK, Stock MC. Handbook of Clinical Anesthesia. Edisi ke 6. Lippincott Williams & Wilkins. 2009:240–241. 18. Morgan GE, Mikhail MS, Murray MJ.Clinical Anesthesiology. Edisi ke 4. Mc Graw Hill. 2005:471, 476–477.