Seorang penderita anemia berat dengan kemungkinan talasemia-? minor kombinasi hemoglobin e

Abstract

Talasemia-?disebabkan olehpenurunan sintesis rantai beta dari globin. Hemoglobin E adalah salah satubentuk hemoglobinopatiyangasam amino glutamatnyadigantikan oleh asam amino lisin pada posisi 26 rantaiglobin ?. Kami melaporkan pasien lelaki 26 tahun dengan keluhan lemas badan. Pada pemeriksaan didapatkananemia berat hipokromik mikrositer, splenomegali, kardiomegali, dan peningkatan kadar HbA2 dan HbF.Dalam perawatan, pasien diberikan tranfusi PRC 1 kolf perhari sampai Hb >10 g/dl. Pembuktian hemoglobinE dengan analisis DNA metode PCR tidak dikerjakan karena biaya. Keluhan lemas badan teratasi dengantransfusi PRC. Pasien ini akan mengalami banyak tranfusi darahdengan akibat kelebihan besi padaorganorgantubuh.[MEDICINA.2016;47(3):58-62]

? Thalassemia is caused by decreasing synthesis of beta globin chains. Hemoglobin E is one ofhemoglobinopathies which glutamic amino acid was replaced by lysine amino acid at 26 ? globin chainposition. We reported a male patient 26 years old with complaintsof weakness. On physical examination wefound severe anemia, hipocromic micrositer, splenomegaly, cardiomegaly, and increasing the level of HbA2and HbF. During treatment, patient has got PRC transfusion one bag a day until Hb >10 g/dl. For provinghemoglobin E with DNA analysis and PCR has not be done due to financial problem. The weaknessimprovedby PRC transfusion. This patient will receive a lot of blood transfusion with its complication is iron overloadin the body.[MEDICINA. 2016;47(3):58-62]