Protein C levels in β-thalassemia major patients in the east Nile delta of Egypt

Hassan, Tamer; Elbehedy, Rabab M.; Youssef, Doaa Mohammed; Amr, Ghada E.

doi:10.1016/s1658-3876(10)50036-0

Cited by 19 publications

(17 citation statements)

References 22 publications

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“…Significant reductions in PC and PS plasma activity and elevation of sEPCR were observed in nonsplenectomized compared with post splenectomy patients. In contrast to our results, Hassan et al reported significantly decreased PC and PS levels in splenectomized β-TM patients compared with controls [15] and Musumeci et al reported that the lowest PC and AT III levels were observed in older splenectomized patients [43]. Natesirinilkul et al found no significant differences in PC and PS levels in splenectomized and nonsplenectomized Hb E/β-thal patients [1].…”

Section: Tablecontrasting

confidence: 99%

“…Previous studies have reported decreased PC and PS activity in β-TM [15,19,21] and Hb E/β-thal [22], and decreased PC in β-TI [23]. We observed a significant decrease of PC and PS activity in α-TI and α + β-thal and the PC deficiency was consistent with Type II abnormalities.…”

Section: Discussionsupporting

confidence: 79%

“…Serum ferritin was inversely correlated with PC antigen levels and positively correlated with TAT. In a study conducted in Egypt, Hassan et al reported that serum ferritin was increased and negatively correlated with PC in β-TM [15]. Toxic non-transferrin-bound iron promotes chronic oxidative stress in thalassemia patients.…”

Section: Discussionmentioning

confidence: 99%

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Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin

Huang¹,

Yuan²,

Deng

et al. 2018

Thrombosis Research

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Background: Thalassemia is characterized by a hypercoagulable state in which the protein C (PC) pathway controls thrombosis. We investigated changes in PC, protein S (PS), antithrombin III (AT III) and soluble endothelial protein C receptor (sEPCR) in thalassemia. Methods: A group of 129 patients with β-thalassemia major (β-TM), β-thalassemia intermedia (β-TI), α-thalassemia intermedia (α-TI) and combined α-/β-thalassemia (α + β-thal) were compared with 32 gender-and age-matched controls. PC, PS, AT III, sEPCR, thrombin-antithrombin complex (TAT), and intercellular adhesion molecule1 (ICAM-1) antigens were measured by enzyme-linked immunosorbent assay. PC, AT III, and PS activity were assayed by substrate chromatography and a prothrombin time (PT)-based free protein S assay. Results: PC deficiency was seen in 95.3% of the patients and PS deficiency was seen in 77.5%. Concomitant reductions in PC and AT III antigen and activity were observed in β-TM, β-TI, and α-TI than in controls (p < 0.005). PC activity was lower in β-TM than in α-TI (p = 0.004). PS antigen was elevated in β-TM (p = 0.011) and sEPCR was elevated in α-TI (p = 0.018). Nonsplenectomized patients had lower PC (p = 0.001) and PS (p = 0.006) and higher sEPCR (p = 0.021) than postsplenectomy patients. Transfusion dependent thalassemia (TDT) patients had lower PC levels (p < 0.005) than those with nontransfusion dependent thalassemia (NTDT). ICAM-1 was increased in patient subgroups (p < 0.001), especially those with splenectomies (p = 0.009), and TAT was increased in all patient subgroups compared with controls (p < 0.001) except for α + β-thal. Conclusions: Deficiencies of anticoagulant proteins and elevated sEPCR contributed to chronic hypercoagulability in these thalassemia patients of Chinese origin. Splenectomy alleviated these alterations in this patient cohort with the median duration since splenectomy of two years. Blood transfusion was not ideal for avoiding thrombosis. 1. Introduction Mutations or deletions of globin genes in thalassemia result in an imbalance of globin synthesis, decreased erythropoiesis, and extravascular hemolysis. Hypercoagulability states are common occurrences in thalassemia patients in southeast Asia, the Mediterranean, and the Middle East regions [1-3]. The prevalence of β-thalassemia is approximately 4.91% and that of α-thalassemia is about 14.13% in the Guangxi province of China, and the various causes of coagulation disorders deserve investigation [4]. Accumulation of unmatched globin chains results in premature destruction of erythrocyte precursors in bone marrow, hemolysis of erythrocytes in the peripheral blood, and the appearance of abnormal red blood cells (RBCs). Loss of deformability impairs passage of RBCs through capillaries [5], and the resulting hemostasis may elicit a procoagulant condition [6]. Other reasons may include endothelial injury, platelet activation, increased plasma microparticles, impaired nitric oxide bioavailability, increased blood oxidants, and phosphatidylserine exposure on the outer ...

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Section: Tablecontrasting

confidence: 99%

Section: Discussionsupporting

confidence: 79%

See 1 more Smart Citation

Alterations of anticoagulant proteins and soluble endothelial protein C receptor in thalassemia patients of Chinese origin

Huang¹,

Yuan²,

Deng

et al. 2018

Thrombosis Research

View full text Add to dashboard Cite

show abstract

“…Decreased levels of protein C and protein S in thalassemic patients have been reported elsewhere. Hassan et al 8 reported decreased levels of protein C and S in their study of 50 children with thalassemia in comparison to the control group. 9 Sipahi et al 10 reported that protein C, protein S, and AT-III were low in 26.2%, 28.6%, and 46.8% of their thalassemic patients, respectively.…”

Section: Discussionmentioning

confidence: 93%

“…Several studies have reported high plasma D-dimers levels in TM patients that are enhanced after splenectomy. 8,17 Cappellini et al 3 reported normal plasma D-dimers levels in TM patients but higher levels in TI patients that were also most pronounced in splenectomized patients. In contrast, Naithani et al 18 reported similar D-dimers levels in b-thalassemic patients and controls.…”

Section: Discussionmentioning

confidence: 99%