Splenectomy and thrombosis: the case of thalassemia intermedia

Taher, Ali T.; Musallam, Khaled M.; Karimi, Mehran; El‐Beshlawy, Amal; Belhoul, Khawla; Daar, Shahina; Saned, Mohamed-SalahEldin; Cesaretti, Claudia; Cappellini, Maria Domenica

doi:10.1111/j.1538-7836.2010.03940.x

Cited by 127 publications

(134 citation statements)

References 41 publications

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“…High nucleated RBC (≥300 × 10 6 /l) and platelet counts (≥500 × 10 9 /l), evidence of PHT and transfusion naivety were the common features associated with higher risk of TEE in splenectomized patients. Acording to this observation, laboratory markers, presence of PHT and transfusion status could be useful to identify early splenectomized TI patients with a high risk for TEE [42]. In addition, it is possible to consider TEE in splenectomized TI patients as a manifestation of a chronic underlying process and not an acute event, based on the evidence that the median time to thrombosis is 8 years, emphasizing the need for a long-term treatment modality for prevention [42].…”

Section: Thrombosis In Thalassemiamentioning

confidence: 99%

“…High nucleated RBC and platelet counts, evidence of PHT and transfusion naivety can be used as markers of an increased risk of developing TEE for splenectomized patients with TI and could be practical in the clinical setting [42].…”

Section: Key Issuesmentioning

confidence: 99%

“…To further illustrate the effect of splenectomy on TEE and to identify high-risk patients in this subgroup, a substudy of the OPTIMAL CARE study examined the characteristics of splenectomized TI patients who had developed TEE [42]. High nucleated RBC (≥300 × 10 6 /l) and platelet counts (≥500 × 10 9 /l), evidence of PHT and transfusion naivety were the common features associated with higher risk of TEE in splenectomized patients.…”

Section: Thrombosis In Thalassemiamentioning

confidence: 99%

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Hypercoagulability in β-thalassemia: a status quo

Cappellini¹,

Poggiali

Taher

et al. 2012

Expert Review of Hematology

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Section: Thrombosis In Thalassemiamentioning

confidence: 99%

Section: Key Issuesmentioning

confidence: 99%

Section: Thrombosis In Thalassemiamentioning

confidence: 99%

See 1 more Smart Citation

Hypercoagulability in β-thalassemia: a status quo

Cappellini¹,

Poggiali

Taher

et al. 2012

Expert Review of Hematology

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“…6 In turn HMC alter the membrane clustering band 3 and enhance the deposition of opsonin autologous immunogobulins and C3 fragments. 7,8 Splenectomy, performed to alleviate anemia in TI patients, may result in severe thrombotic episodes 9,10 and may cause a rise of pro-thrombotic circulating microparticles (MP). 11 The composition and pathogenic roles of MP have been extensively studied in various diseases, such as ischemia, diabetes and atherosclerosis, revealing complex pathogenic roles in modulating nitric oxide and prostacyclin production, stimulating cytokine release, inducing tissue factor expression, as well as monocyte chemotaxis and adherence to the endothelium.…”

Section: Introductionmentioning

confidence: 99%

Thalassemic erythrocytes release microparticles loaded with hemichromes by redox activation of p72Syk kinase

Ferru

Pantaleo

Carta³

et al. 2013

Haematologica

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© F e r r a t a S t o r t i F o u n d a t i o ndifferent genetic and clinical status provided new insights into the pathogenic role of circulating MP and possible interventions to control their amount in thalassemia. MethodsUnless otherwise stated, all materials were obtained from Sigma-Aldrich, St. Louis, MO, USA. Additional information about the methods are provided in the Online Supplementary Data. Treatment of red blood cellsVenous blood was drawn from 12 healthy individuals, eight non-splenectomized TI patients and six splenectomized TI patients. All subjects provided written, informed consent before entering the study. The study was approved by the local Ethics Committee and conducted in accordance with Good Clinical Practice guidelines and the Declaration of Helsinki.Clinical analyses were performed by routine laboratory tests at the Policlinico Hospital (Milan, Italy). Blood anti-coagulated with heparin was stored in citrate-phosphate-dextrose with adenine (CPDA-1) prior to its use. RBC were washed three times with phosphate-buffered saline (PBS: 137mM NaCl, 2.7mM KCl, 8.1mM K 2 HPO 4 , 1.5mM KH 2 PO 4 , pH 7.4) containing glucose 5 mM to obtain packed RBC.To stimulate HMC formation, RBC from healthy donors were suspended at a hematocrit of 30% and incubated with different concentrations (0, 0.25, 0.5, 1, 1.5, 2 mM) of phenylhydrazine at 37 °C for 4 h. To test the antioxidant effect in MP release we incubated 200 μM of 2-mercaptoethanol in the presence of 1 mM phenylhydrazine. When necessary, RBC were pretreated with Syk kinase inhibitors (10 μM Syk inhibitors II and 10 μM Syk inhibitors IV, Calbiochem, Darmstadt, Germany), for 1 h at 37 °C in the dark. Each reaction was terminated by three washes with PBS-glucose. For all protocols described, untreated controls were processed identically except that the stimulant/inducer was omitted from the incubation. Red blood cell membrane preparationStandard hypotonic membranes were prepared, as previously described, 20 and stored frozen at -80°C until use. Membrane protein content was quantified using the DC Protein assay (Biorad, Hercules, CA, USA). Analysis of microparticlesThe MP in plasma were analyzed by flow cytometry using a modification of a previously described method:22 25 μL of plasma diluted 1:1 with PBS-glucose 5mM were analyzed using anti-CD41 (BD, Franklin Lakes, NJ, USA) and anti-glycophorin-A (Dako, Denmark), both diluted 1:10. Assay of hemichromesHMC were quantified by measuring heme absorbance (Abs) using the following equation:HMC= -133xAbs577 -114xAbs630 + 233xAbs560, and expressed as nMoles/mL of solubilized membranes. 23 Microparticle isolationTo induce MP release in vitro, RBC from each volunteer and phenylhydrazine-treated RBC in PBS (30% hematocrit) were incubated as previously described. 24 Electrophoresis and immunoblottingMembrane and MP proteins were solubilized in Laemmli buffer 25 under reducing [2% (w/v) dithiothreitol] or non-reducing conditions at a volume ratio of 1:1. Sodium dodecylsulfate polyacrylamide gel electropho...

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“…The phenomenon was seen more common in thalassemia intermedia patients than in regularly transfused thalassemia major patients. Among thalassemia major patients, the prevalence is higher in splenectomized patients [4,5].…”

Section: Introductionmentioning

confidence: 99%

The Level of Natural Anticoagulants in Transfusion Dependent Thalassemia Patients in Kelantan, Northeastern Malaysia

Rosnah

Mn²,

Shafini³

et al. 2014

J Hematol Thrombo Dis

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The association of the severe form of thalassemia with complications of blood transfusion, iron overload, bony deformity and gall stone is well described. Currently, due to improvement in the way of managing thalassemia, most of thalassemia patients survive longer. However more new complications had been observed.Increased incidence of thromboembolism among thalassemia patients had triggered various studies done to determine hypercoagulable state among thalassemia patients. Several factors for the hypercoagulable state had been identified such as RBC membrane disruption, chronic platelet activation and defect in coagulation pathway. This study was carried out to determine the level of Protein C, protein S, free protein S and antithrombin among thalassemia patients and to compare the level of Protein C, protein S, free Protein S and antithrombin level between thalassemia patients and healthy control. This was a case control study done at Hospital Universiti Sains Malaysia. Thirty six thalassemia patients who came for regular blood transfusion and 20 healthy blood donors for normal control were recruited. Blood samples were collected and analyzed for Protein C, protein S, free protein S and antithrombin using ACL Elite Pro. The result showed mean Protein C and free protein S levels were significantly lower (54.8 ± 13.2% and 70.0 ± 12.1% respectively) in thalassemia patients compared to age-matched normal control (94.1 ± 16.3% and 99.8 ± 17.3% respectively), whereas mean total protein S (54.8 ± 13.2% and 94.1 ± 16.3%; respectively) and antithrombin (70.0 ± 12.1% and 99.8 ± 17.3%; respectively) level were similar. In conclusion there was a significantly decreased Protein C and free protein S in thalassemia patients which might suggests hypercoagulable state in thalassemia patients. Since there are a lot of similarities in finding from other studies, we believe that many more study to look for other parameters contributing to hypercoagulable state in thalassemia patients is needed.

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Splenectomy and thrombosis: the case of thalassemia intermedia

Cited by 127 publications

References 41 publications

Hypercoagulability in β-thalassemia: a status quo

Hypercoagulability in β-thalassemia: a status quo

Thalassemic erythrocytes release microparticles loaded with hemichromes by redox activation of p72Syk kinase

The Level of Natural Anticoagulants in Transfusion Dependent Thalassemia Patients in Kelantan, Northeastern Malaysia

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