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vol. 15
Basic research

The effect of splenectomy on complement regulatory proteins in erythrocytes in β-thalassemia major

Ayşegül Uğur Kurtoğllu, Belkıs Koçtekin, Erdal Kurtoğlu, Mustafa Yildiz

Arch Med Sci 2019; 15, 1: 191–195
Online publish date: 2018/12/30
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Hemolysis due to ineffective erythropoiesis is a serious problem β-thalassemia major (β-TM) patients. The role of complement system in the etiopathogenesis of hemolysis observed in β-TM were released. Hemolysis induced by activation of complement system is prevented by complement regulatory proteins. Decay accelerating factor (CD55), membrane inhibitor of reactive lysis (CD59), and complement reception 1 (CR1, CD35) are among these proteins. The absence of these proteins thus accounts for the increased susceptibility of erythrocytes to complement lysis. Splenomegaly and hypersplenism are common complications among thalassemia major patients necessitating splenectomy.

Material and methods
In this study we investigated how splenectomy effects complement regulatory system in erythrocytes. We analysed CD35, CD55, and CD59 levels on erythrocytes in β-TM by flow cytometry.

The overall mean percentage of CD55 and CD35 positive RBCs of group 1 (22 β-TM with splenectomy) was significantly lower than group 2 (23 β-TM without splenectomy) and group 3 (healthy controls) (p < 0.05). The overall mean percentage CD59 positive RBCs of patients was no significantly different in all groups. The levels of CD35 and CD55 expression on the erythrocytes of splenectomized patients was significantly lower than non-splenectomized patients (p < 0.05).

Increased erythrocyte destruction and iron deposition in organs due to deficiency of these regulatory proteins may be the underlying mechanism of organ damage developing in β-TM patients.


splenectomy, β-thalassemia, complement regulatory proteins

Guvenc B, Canataroglu A, Unsal C, et al. β-Thalassemia mutations and hemoglobinopathies in Adana, Turkey: results from a single center study. Arch Med Sci 2012; 8: 411-4.
Gardenghi S, Marongiu MF, Ramos P, et al. Ineffective erythropoiesis in beta-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation ferroportin. Blood 2007; 109: 5027-35.
Darzi AA, Kamali S, Khakzad M. Influence of splenectomy on immunoglobulins and complement components in major thalassemia. Caspian J Intern Med 2015; 6: 30-3.
Ruiz-Delgado GJ, Vázquez-Garza E, Méndez-Ramírez N, Gómez-Almaguer D. Abnormalities in the expression of CD55 and CD59 surface molecules on peripheral blood cells are not specific to paroxysmal nocturnal hemoglobinuria. Hematology 2009; 14: 33-7.
McGreal E, Gasque P. Structure-function studies of the receptors for complement C1q. Biochem Soc Trans 2002; 30: 1010-4.
Obaid JM, Abo El-Nazar SY, Ghanem AM, El-Hadidi AS, Mersal BH. Expression of CD55 on red blood cells of β-thalassemia patients. Hemoglobin 2014; 38: 339-44.
Al-Salem AH. splenectomy for children with thalassemia: total or partial splenectomy, open or laparoscopic splenectomy. J Pediatr Haematol Oncol 2016; 38: 1-4.
Di Sabatino A, Carsetti R, Corazza GR. Post-splenectomy and hyposplenic states. Lancet 2011; 378: 86-97.
Edgren G, Almqvist R, Hartman M, Utter GH. Splenectomy and the risk of sepsis: a population-based cohort study. Ann Surg 2014; 260: 1081-7.
Martin A, Thompson AA. Thalassemias. Pediatr Clin North Am 2013; 60: 1383-91.
Sayani FA, Kwiatkowski JL. Increasing prevalence of thalassemia in America: implications for primary care. Ann Med 2015; 47: 592-604.
Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood 2011; 118: 3479-88.
Brodsky RA. Complement in hemolytic anemia. Blood 2015; 126: 2459-65.
DeZern AE, Brodsky RA. Paroxysmal nocturnal hemoglobinuria: a complement-mediated hemolytic anemia. Hematol Oncol Clin North Am 2015; 29: 479-94.
Lintner KE, Wu YL, Yang Y, Spencer CH, Hauptmann G, Hebert LA. Early components of the complement classical activation pathway in human systemic autoimmune diseases. Front Immunol 2016; 7: 36.
Krych-Goldberg M, Atkinson JP. Structure-function relationships of complement receptor type 1. Immunol Rev 2001; 180: 112-22.
Pu JJ, Brodsky RA. Paroxysmal nocturnal hemoglobinuria from bench to bedside. Clin Transl Sci 2011; 4: 219-24.
Kahng J, Kim Y, Kim JO, Koh K, Lee JW, Han K. A novel marker for screening paroxysmal nocturnal hemoglobinuria using routine complete blood count and cell population data. Ann Lab Med 2015; 35: 35-40.
Gupta PK, Charan VD, Kumar H. PNH revisited: clinical profile, laboratory diagnosis and follow-up. Indian J Pathol Microbiol 2009; 52: 38-41.
Li Y, Lu L, Li J. Topological structures and membrane nanostructures of erythrocytes after splenectomy in hereditary spherocytosis patients via atomic force microscopy. Cell Biochem Biophys 2016; 74: 365-71.
Ibrahim HA, Fouda MI, Yahya RS, Abousamra NK, Abd Elazim RA. Erythrocyte phosphatidylserine exposure in beta-thalassemia. Lab Hematol 2014; 20: 9-14.
Lal A, Gomez E, Calloway C. Increased mitochondrial DNA deletions and copy number in transfusion-dependent thalassemia. JCI Insight 2016; 1. pii: e88150.
Malik D, Hsu T, Falatoonzadeh P, et al. Human retinal transmitochondrial cybrids with J or H mtDNA haplogroups respond differently to ultraviolet radiation: implications for retinal diseases. PLoS One 2014; 9: e99003.
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