ĐẶC ĐIỂM LÂM SÀNG VÀ HUYẾT HỌC BỆNH THALASSEMIA Ở TRẺ EM TẠI BỆNH VIỆN NHI THANH HÓA

Abstract

Objective: To describe the clinical and hematological characteristics of thalassemia in children at Thanh Hoa Children's Hospital. Subjects and   methods: Cross-sectional description  of 170 patients  were  diagnosed  with  thalassemia  at  Thanh Hoa Children's Hospital from 2021-2022. Results: The majority of patients in the age range < 5 years (48.8%); average age 7.3 ± 3.5 years. There are 3 disease types: HbH, β-thalassemia and HbE/β-thalassemia with the rate of 7.6%, 37.1% and 55.3% respectively. The severity of the disease was mainly intermediate (66.5%) and severe (32.4%), the rest was mild (1.1%). Clinical manifestations: 100% of anemia mainly moderate/severe (98.2%), 74.1% jaundice, 59.4% splenomegaly, 54,1% skull/face deformity, 50% tanning, 39.4% hepatomegaly and 17.6% physical retardation. There were significant differences in mean pre-transfusion Hb, number of blood transfusions/year among disease types. Most patients had iron overload with moderate (38.2%) and severe (35.3%) serum ferritin levels. Conclusion: The patient has clinical features of β-thal, HbE/β-thal and HbH at intermediate and severe levels; hematologic characteristics with low mean pre-transfusion Hb, moderate and severe iron overload complications.