The Clinical Approach toward Hereditary Persistence of Fetal Hemoglobin: A Case Report
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Abstract
Fetal hemoglobin is the principal hemoglobin in the human fetus, and the adult levels of fetal hemoglobin (HbF) are less than 1% of total hemoglobin. A steady increase of HbF in patients with hereditary persistence of fetal hemoglobin (HPFH) is associated with complications. The present report describes HPFH in a 26-year-old man with emphasis on its hemoglobin electrophoresis. The patient was admitted with complaints of recurrent weakness and lethargy, weight loss, abdominal pain, and dyspepsia. Splenectomy was planned due to massive splenomegaly and gastrointestinal complications. Ultimately, electrophoresis confirmed the diagnosis of HPFH.
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9. Sankaran VG. Targeted therapeutic strategies for fetal hemoglobin induction. Hematology Am Soc Hematol Educ Program. 2011;2011:459-65.
10. Karimi M, Musallam KM, Cappellini MD, Daar S, El-Beshlawy A, Belhoul K, et al. Risk factors for pulmonary hypertension in patients with β thalassemia intermedia. Eur f Int Med. 2011;22(6):607-10.
11. Mansoori H, Asad S, Rashid A, Karim F. Delta beta thalassemia: a rare hemoglobin variant. Blood Res. 2016;51(3):213-4.
12. Lai Z-S, Yeh T-K, Chou Y-C, Hsu T, Lu C-T, Kung F-C, et al. Potent and orally active purine-based fetal hemoglobin inducers for treating β-thalassemia and sickle cell disease. Eur J Med Chem. 2021;209:112938.
13. Sharma DC, Singhal S, Woike P, Rai S, Yadav M, Gaur R. Hereditary persistence of fetal hemoglobin. Asian J Transfus Sci. 2020;14(2):185-6.
14. Sajadpour Z, Amini-Farsani Z, Motovali-Bashi M, Yadollahi M, Khosravi-Farsani N. Association between Different Polymorphic Markers and β-Thalassemia Intermedia in Central Iran. Hemoglobin. 2020;44(1):27-30.
15. Lee HS. Effects of Serum Ferritin and White Blood Cell on Overweight and Obesity in South Korean Adults. Indian Journal of Public Health Research & Development. 2019;10(11).
16. Fromhold-Treu S, Lamprecht G. [Gastrointestinal causes of weight loss: clinical presentation, diagnostic workup and therapy]. Dtsch Med Wochenschr. 2016;141(4):253-60.
17. Djiambou-Nganjeu H. Relationship Between Portal HTN and Cirrhosis as a Cause for Diabetes. J Trans Int Me. 2019;7(2):79-83.
18. Vick EJ, Patel K, Prouet P, Martin MG. Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy. Blood Advances. 2017;1(12):779-91.
19. Alavi S, Arabi N. Case 1: An 18-month-old female infant with pancytopenia and hepatosplenomegaly. Paediatr Child Health. 2014;19(7):347-9.
2. Silva DGH, Junior EB, De Almeida EA, Bonini-Domingos CR. Oxidative stress in sickle cell disease: an overview of erythrocyte redox metabolism and current antioxidant therapeutic strategies. Free Radic Biol Med. 2013;65:1101-9.
3. Torres Lde S, da Silva DG, Belini Junior E, de Almeida EA, Lobo CL, Cançado RD, et al. The influence of hydroxyurea on oxidative stress in sickle cell anemia. Rev Bras Hematol Hemoter. 2012;34(6):421-5.
4. Sokolova A, Mararenko A, Rozin A, Podrumar A, Gotlieb V. Hereditary persistence of hemoglobin F is protective against red cell sickling. A case report and brief review. Hematol Oncol Stem Cell Ther. 2019;12(4):215-9.
5. Godown J, Lu JC, Beaton A, Sable C, Mirembe G, Sanya R, Aliku T, Yu S, Lwabi P, Webb CL, Ensing GJ. Handheld echocardiography versus auscultation for detection of rheumatic heart disease. Pediatrics. 2015 Apr;135(4):e939-44. doi: 10.1542/peds.2014-2774. Epub 2015 Mar 16. PMID: 25780068..
6. Mosca A, Paleari R, Leone D, Ivaldi G. The relevance of hemoglobin F measurement in the diagnosis of thalassemias and related hemoglobinopathies. Clin Biochem. 2009;42(18):1797-801.
7. Ghani SNAM. Haematological and molecular characterisation of high haemoglobin F among anaemic patients in Hospital Universiti Sains Malaysia: Pusat Pengajian Sains Kesihatan, Universiti Sains Malaysia; 2020.
8. Musallam KM, Taher AT, Cappellini MD, Sankaran VG. Clinical experience with fetal hemoglobin induction therapy in patients with β-thalassemia. Blood. 2013;121(12):2199-212; quiz 372.
9. Sankaran VG. Targeted therapeutic strategies for fetal hemoglobin induction. Hematology Am Soc Hematol Educ Program. 2011;2011:459-65.
10. Karimi M, Musallam KM, Cappellini MD, Daar S, El-Beshlawy A, Belhoul K, et al. Risk factors for pulmonary hypertension in patients with β thalassemia intermedia. Eur f Int Med. 2011;22(6):607-10.
11. Mansoori H, Asad S, Rashid A, Karim F. Delta beta thalassemia: a rare hemoglobin variant. Blood Res. 2016;51(3):213-4.
12. Lai Z-S, Yeh T-K, Chou Y-C, Hsu T, Lu C-T, Kung F-C, et al. Potent and orally active purine-based fetal hemoglobin inducers for treating β-thalassemia and sickle cell disease. Eur J Med Chem. 2021;209:112938.
13. Sharma DC, Singhal S, Woike P, Rai S, Yadav M, Gaur R. Hereditary persistence of fetal hemoglobin. Asian J Transfus Sci. 2020;14(2):185-6.
14. Sajadpour Z, Amini-Farsani Z, Motovali-Bashi M, Yadollahi M, Khosravi-Farsani N. Association between Different Polymorphic Markers and β-Thalassemia Intermedia in Central Iran. Hemoglobin. 2020;44(1):27-30.
15. Lee HS. Effects of Serum Ferritin and White Blood Cell on Overweight and Obesity in South Korean Adults. Indian Journal of Public Health Research & Development. 2019;10(11).
16. Fromhold-Treu S, Lamprecht G. [Gastrointestinal causes of weight loss: clinical presentation, diagnostic workup and therapy]. Dtsch Med Wochenschr. 2016;141(4):253-60.
17. Djiambou-Nganjeu H. Relationship Between Portal HTN and Cirrhosis as a Cause for Diabetes. J Trans Int Me. 2019;7(2):79-83.
18. Vick EJ, Patel K, Prouet P, Martin MG. Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy. Blood Advances. 2017;1(12):779-91.
19. Alavi S, Arabi N. Case 1: An 18-month-old female infant with pancytopenia and hepatosplenomegaly. Paediatr Child Health. 2014;19(7):347-9.
| Files | ||
| Issue | Vol 21 No 3 (2022) | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/ijaai.v21i3.9810 | |
| Keywords | ||
| Blood protein electrophoresis Fetal hemoglobin Hemoglobinopathies | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Ghaderi A, Bakhtiari T, Jokar S, Eshraghi A. The Clinical Approach toward Hereditary Persistence of Fetal Hemoglobin: A Case Report. Iran J Allergy Asthma Immunol. 2022;21(3):364-368.
