Hemoglobin C Disorder in Anemic Patients Referred to the National Center for Thalassemia and Genetic Counseling in Damascus

Aya Ahmad; Karam Fattoum; Wael Imam; Mhd Yasser Mukhalalaty; Musab Murad; Faizah Ali  Al Quobaili

doi:10.18502/ijhoscr.v18i2.15376

Aya Ahmad Department of Pharmacy, Al-Sham Private University, AL-Tall Damascus, Syria
Karam Fattoum Department of Pharmacy, Al-Sham Private University, AL-Tall Damascus, Syria
Wael Imam Department of Pharmacy, Al-Sham Private University, AL-Tall Damascus, Syria
Mhd Yasser Mukhalalaty National Center for Thalassemia and Genetic Counseling, Damascus, Syria
Musab Murad Department of Pharmacy, Al-Sham Private University, AL-Tall Damascus, Syria
Faizah Ali Al Quobaili Department of Pharmacy, Al-Sham Private University, AL-Tall Damascus, Syria

DOI: https://doi.org/10.18502/ijhoscr.v18i2.15376

Keywords: Blood transfusion; Hemoglobin S (HbS); Hemolytic anemia; Sex

Abstract

Background: Hemoglobinopathies are common inherited blood disorders in our Mediterranean area. The main structural hemoglobin variants are hemoglobin S and hemoglobin C, due to their prevalence.

We conducted this retrospective study to investigate and characterize hemoglobin C patients referred to the National Center for Thalassemia and Genetic Counseling and the management of hemoglobin C disease in Damascus.

Materials and Methods: The study included patients referred to the National Center for Thalassemia and Genetic Counseling in Damascus between 2000 and 2022 for hemoglobin C detection. Gender, age, geographical origin, hemoglobin electrophoresis profile, and blood transfusion were considered for hemoglobin C patient classification. Blood transfusion in five consecutive years and linear regression with hemoglobin S and C values were determined.

Results: 30 (14 males and 16 females) out of 624 patients between 3 and 46 years old (mean ± SD: 17.3 ± 9.7 years) showed hemoglobin C disease. Only eight patients (one male and seven females) received blood transfusions, and the remaining patients (13 males and 9 females) did not receive any transfusion. Only one patient with 100% hemoglobin C was detected; 19 showed HbSC, and 10 had HbAC. There was a significant correlation between hemoglobin S and geographical origin (P-value=0).

Conclusion: A Homozygote hemoglobin C patient has mild hemolytic anemia, whereas the hemoglobin C 100% patient has only a one-time blood transfusion (he was 17 years old) in our study. The inherited combination of hemoglobin C and S is less severe than hemoglobin S alone. There is a significant relationship between hemoglobin S and geographical origin (p-value=0).