<![CDATA[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
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