Incidence, Risk Factors, and Outcome of Portal Vein Thrombosis After Laparoscopic-Assisted Splenectomy in <i>β</i>-Thalassemia Patients: A Prospective Exploratory Study

Alexakis, N.; Dardamanis, Dimitrios; Albanopoulos, K; Ptohis, Nikolaos; Skalistira, M.; Karagiorga, Markisia; Zografos, George; Leandros, Emmanuel

doi:10.1089/lap.2012.0268

Cited by 16 publications

(10 citation statements)

References 22 publications

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“…A small series of patients with b-thalassemia who underwent splenectomy reported an 8.3% incidence of postsplenectomy VTE, 34 a difference likely caused by the higher burden of hypercoagulability in patients with b-thalassemia compared with ITP.…”

Section: Discussionmentioning

confidence: 99%

Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia

Boyle¹,

White

Brunson³

et al. 2013

Blood

174

148

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• After splenectomy, patients with ITP have a higher risk of venous thrombosis and sepsis than patients with ITP who do not undergo splenectomy.Patients with immune thrombocytopenia (ITP) who relapse after an initial trial of corticosteroid treatment present a therapeutic challenge. Current guidelines recommend consideration of splenectomy, despite the known risks associated with surgery and the postsplenectomy state. To better define these risks, we identified a cohort of 9976 patients with ITP, 1762 of whom underwent splenectomy. The cumulative incidence of abdominal venous thromboembolism (AbVTE) was 1.6% compared with 1% in patients who did not undergo splenectomy; venous thromboembolism (VTE) (deep venous thrombosis and pulmonary embolus) after splenectomy was 4.3% compared with 1.7% in patients who did not undergo splenectomy. There was increased risk of AbVTE early (<90 days; hazard ratio [HR] 5.4 [confidence interval (CI), 2.3-12.5]), but not late ( ‡90 days; HR 1.5 [CI, 0.9-2.6]) after splenectomy. There was increased risk of VTE both early (HR 5.2 [CI, 3.2-8.5]) and late (HR 2.7 [CI, 1.9-3.8]) after splenectomy. The cumulative incidence of sepsis was 11.1% among the ITP patients who underwent splenectomy and 10.1% among the patients who did not. Splenectomy was associated with a higher adjusted risk of sepsis, both early (HR 3.3 [CI, 2.4-4.6]) and late (HR 1.6 or 3.1, depending on comorbidities). We conclude that ITP patients post splenectomy are at increased risk for AbVTE, VTE, and sepsis. (Blood. 2013;121(23):4782-4790)

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Section: Discussionmentioning

confidence: 99%

Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia

Boyle¹,

White

Brunson³

et al. 2013

Blood

174

148

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“…Тем не менее тромбозы у детей с гемолитическими анемиями встречаются нечасто и в основном после оперативного вмешательства [18,28,29], однако уже в детском возрасте мы можем наблюдать развитие дисфункции эндотелия -изменения в концентрациях тромбомодулина и эндотелина-1.…”

Section: результаты исследованияunclassified

Endothelial dysfunction in patients with hereditary spherocytosis and b-thalassemia

Чуйко

Серёгина

Vuimo

et al. 2021

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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Patients with hereditary spherocytosis and b-Thalassemia are characterized by the increased risk of thrombosis. The early manifestation of thrombotic complications can occur even in childhood especially after surgery. Hypercoagulability can be associated with endothelial dysfunction. The aim of this study was to investigate the hemostatic state and endothelial function in children with hereditary spherocytosis and b-thalassemia. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The hemostatic status of 18 children (10 boys and 8 girls from 1 to 13 years) with hereditary spherocytosis and of 8 children (4 boys and 4 girls from 3 to 8 years) with b-thalassemia was assessed using clotting times (activated partial thromboplastin time – APTT, thrombin time – TT, prothrombin time PT), fibrinogen levels and markers of endothelium dysfunction: endothelin-1 and thrombomodulin levels. Patients with hereditary spherocytosis were divided into 2 groups: during the hemolytic crisis (11 patients) and without the hemolytic crisis (7 patients). Patients with b-Thalassemia were divided into 3 groups: b-thalassemia major, b-thalassemia intermedia and b-thalassemia minor. APTT, TT and PT were not changed significantly between groups. We find the decreased fibrinogen levels in patients with severe condition: in hereditary spherocytosis patients during hemolytic crisis (1.9 ± 0.3 ng/ml with normal range 2–3.9 ng/ml) and in b-thalassemia major patients (1.8 ± 0.3 ng/ml with normal range 2–3.9 ng/ml). This could be caused by consumption of fibrinogen during acute hemolysis. The Thrombomodulin levels were increased in all hereditary spherocytosis patients, but median value was higher in group with hemolytic crisis (6665 pg/ml vs 5976 pg/ml with ormal value 275–909 pg/ml) indicating endothelium dysfunction and activation of blood clotting. In b-thalassemia patients Thrombomodulin levels were more elevated in b-thalassemia major and b-thalassemia intermedia (6389 ± 537 pg/ml и 6804 ± 120 pg/ml) compared to b-thalassemia minor (2727 ± 213 pg/ml) which is still higher than normal range. Endothelin-1 levels were elevated on 55% with hereditary spherocytosis patients during crisis vs 43% without. In general Endothelin-1 levels were more elevated in b-thalassemia patients (were normal in b-thalassemia minor) vs hereditary spherocytosis patients (2.33 ± 2.89 fmol/ml vs 0.95 ± 0.35 fmol/ml). Thrombomodulin and endothelin-1 levels revealed endothelium dysfunction in children with hemolysis. More dramatic changes observed in severe condition: in hereditary spherocytosis patients during hemolytic crisis and in b-thalassemia major and b-thalassemia intermedia patients.

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“…Portal vein thrombosis, which is considered as venous thrombosis of an unusual site, is more commonly reported in patients with beta-thalassemia disease [30][31][32][33][34] with the odds ratio of 3.5 [31]. Patients with acute portal vein thrombosis usually present with symptoms and signs of portal hypertension of bowel ischemia, i.e., abdominal pain and distension, fever, nausea/vomiting, rectal hemorrhage, and splenomegaly.…”

Section: Venous Thromboembolismmentioning

confidence: 99%

Thromboembolism in Beta-Thalassemia Disease

Natesirinilkul¹

2020

Beta Thalassemia

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Thalassemia disease is a common inherited hemolytic anemia frequently found in several parts of the world, especially in the Mediterranean and some Asian countries. Besides the complications of secondary hemochromatosis from regular red blood cell (RBC) transfusion and increased gastrointestinal absorption of iron, thromboembolism (TE) is one of the common long-term complications of beta-thalassemia disease, particularly in patients with non-transfusion-dependent thalassemia (NTDT), which is commonly seen after the second decade of life. The risk factors of TE in beta-thalassemia disease including exposure of phosphatidylserine of abnormal RBCs, increase of platelet activation and aggregation, elevation of endothelial microparticles and increased endothelial activation, decreased nitric oxide (NO) secondary to hemolysis, rise of platelet count and nucleated RBCs after splenectomy, organ dysfunction caused by hemochromatosis, and thrombophilia such as natural anticoagulant deficiencies leading to hypercoagulable state. The understanding of the pathophysiology would result in effective prevention of this complication of beta-thalassemia disease.

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Incidence, Risk Factors, and Outcome of Portal Vein Thrombosis After Laparoscopic-Assisted Splenectomy in β-Thalassemia Patients: A Prospective Exploratory Study

Abstract: Patients with β-thalassemia who undergo laparoscopic-assisted splenectomy are at high risk of postoperative PVT. Close postoperative surveillance and aggressive coagulation prophylaxis are needed in these patients. Larger studies are required to confirm the present findings.

Cited by 16 publications

References 22 publications

Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia

Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia

Endothelial dysfunction in patients with hereditary spherocytosis and b-thalassemia

Thromboembolism in Beta-Thalassemia Disease

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