Original Article

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

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).

1. Desai P, Sayani F, Lanzkron S. Thalassemia, sickle cell disease, and other hemoglobinopathies. In: Cuker A, Altman J, Gerds A, Wun T. (eds). American Society of Hematology Self-Assessment Program, 7th edn. American Society of Hematology: USA, 2019, pp 161-186.
2. Hoffbrand V, Moss P. (eds). Hoffbrand’s Essential Haematology, 7th edn. John Wiley & Sons Ltd; West Sussex, UK, 2016.
3. Benz, E. Disorders of Hemoglobin. In: Longo, D. (eds). Harrison’s Hematology and Oncology, 3rd edn. McGraw-Hill: Boston, USA , 2017, pp 81–94.
4. Bates, I. & Bain, B. J. Approach to the diagnosis and classification of blood diseases. In Bain, B. J., Bates, I., Laffan, M. A. & Lewis, S. M (eds) Dacie and Lewis Practical Haematology, 11th edn. Churchill Livingstone: London, UK , 2017, pp 549–562.
5. Nayak, R. Rapid Review of Hematology. JAYPEE: India, 2014.
6. Forget BG, Bunn HF. Classification of the Disorders of Hemoglobin. Cold Spring Harb Perspect Med. 2013;3(2):a011684.
7. Hatton, C. S. R., Hughes-Jones, N. C., Hay, D. & Keeling, D. M. Haematology, 9th edn. John Wiley & Sons: Chichester, UK, 2013
8. Ouzzif Z, El Maataoui A, Oukhedda N, et al. Hemoglobinosis C in Morocco : A report of 111 cas. Tunis Med. 2017;95(12):229-233.
9. Piel FB, Howes RE, Patil AP, et al. The distribution of hemoglobin C and its prevalence in newborns in Africa. Sci Rep. 2013;3:1671.
10. Jorge SE., Ribeiro DM, Santos MNN & de Fátima Sonati, M. Hemoglobin: Structure, Synthesis and Oxygen Transport. In Costa, F. F. & Conran, N (eds). Sickle Cell Anemia: From Basic Science to Clinical Practice, Springer International Publishing: Sao Paulo, Brazil, 2016, pp 1–22.
11. Al-Salem, A. Medical and Surgical Complications of Sickle Cell Anemia. Springer International Publishing: Saudi Arabia, 2016
12. Piel FB, Patil AP, Howes RE, et al. Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun. 2010;1:104.
13. Murphy WG. The sex difference in haemoglobin levels in adults — Mechanisms, causes, and consequences. Blood Rev. 2014;28(2):41-7.
14. Kundrapu S, Janaki N, Meyerson HJ. Compound Heterozygosity for Hb D-Ibadan ( HBB : c.263C>A) and Hb C ( HBB : c.19G>A). Hemoglobin. 2018;42(4):269-271.
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IssueVol 18 No 2 (2024) QRcode
SectionOriginal Article(s)
DOI https://doi.org/10.18502/ijhoscr.v18i2.15376
Keywords
Blood transfusion Hemoglobin S (HbS Hemolytic anemia Sex
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Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Ahmad A, Fattoum K, Imam W, Mukhalalaty MY, Murad M, Al Quobaili F. Hemoglobin C Disorder in Anemic Patients Referred to the National Center for Thalassemia and Genetic Counseling in Damascus. Int J Hematol Oncol Stem Cell Res. 2024;18(2):183-191.