Evaluation of Clinical, Laboratory Characteristics of Hemoglobin H Disease Patients from 2002 to 2022 Referred to Thalassemia Center, Bu-Ali Sina Hospital in Sari North of Iran

Abstract

Abstract Background Alpha Thalassemia is a common hereditary anemia which results from decreased alpha-globin production. The clinical manifestations of these genetic abnormalities range from a silent carrier state, to fatal hydrops fetalis. Hemoglobin H disease (HbH disease) is type of α-thalassemia which is affect three of the four α-globin genes. Methods This study was conducted on all patients with hemoglobinopathy H (according to electrophoresis) registered in the thalassemia center of Bo-Ali Sina Hospital in Sari. 46 patients were identified during 20 years. After considering the inclusion criteria, demographic information, clinical information, and laboratory information were collected. The data were entered into SPSS version 21 software to perform statistical analysis. Results The results of the study showed that most of the patients were female and in the younger age group. Laboratory findings of patients, average hemoglobin and blood parameters were reported. The results showed that the most common symptoms among the patients were pallor and fatigue. Jaundice was the most common complication, followed by pneumonia and gallstones. It also showed that 32% of patients dependent on blood transfusion. Conclusion The average hemoglobin H level was significantly higher in individuals with fatigue and pallor compared to those without these signs. The most reported complications were jaundice. Patients with higher HbH levels significantly experienced more occurrences of cholelithiasis.