Hypercoagulability and thrombotic complications in hemolytic anemias

Ataga, Kenneth I.

doi:10.3324/haematol.2009.013672

Cited by 150 publications

(122 citation statements)

References 41 publications

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“…ETP and the peak height are the most used to date to assess clinical relevance. The higher ETP and higher peak height we observed in the presence of thrombomodulin are consistent with the high risk of thrombosis observed in SCD patients [2,3,5,19]. Lag time and velocity index, two other parameters of the thrombin generation curve, could at least help to identify the underlying pathophysiology of abnormal features.…”

Section: Discussionsupporting

confidence: 86%

Thrombin generation reveals high procoagulant potential in the plasma of sickle cell disease children

et al. 2011

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Changes in several components of the clotting system are well documented in sickle cell disease (SCD) patients. However, whether the global hemostatic potential of these patients is altered is still unclear. Calibrated automated thrombogram 1 method of thrombin generation (TG) was used to characterize the hemostatic potential of 83 SCD children (75 SS, 6 SC, and 2 Sb thal ) at steady-state as compared with 50 controls of the same range of age. TG was triggered using 1 pM tissue factor and 4 lM phospholipids with and without thrombomodulin. Thirteen SCD children were also evaluated during vaso-occlusive crisis. Protein C activity, free protein S and D-dimers levels were measured in parallel. SCD patients showed higher rates of thrombin formation, higher thrombin peak height (with and without thrombomodulin), and higher endogenous thrombin potential (ETP) than controls in the presence of thrombomodulin. Reduction of ETP (RETP) in the presence of thrombomodulin was lower in SCD group compared with controls and correlated both with protein C and protein S levels. ETP, RETP, peak height, and velocity index of TG correlated with D-dimers. Compound heterozygous patients showed an intermediate hemostatic phenotype at steady-state. No significant difference was observed when comparing TG parameters during vaso-occlusive crisis to those obtained at steady-state in the same patients. The global hemostatic potential is increased and reflects the hypercoagulable state of SCD patients even at steady-state. The relevance of this finding with respect to the risk of thrombotic complications of the disease needs further investigation. Am. J. Hematol. 87:145-149, 2012. V

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Section: Discussionsupporting

confidence: 86%

Thrombin generation reveals high procoagulant potential in the plasma of sickle cell disease children

et al. 2011

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“…Haemolysis is generally thought to cause thromboembolism due to the abnormal exposure of phosphatidylserine following red cell destruction, which promotes coagulation [18]. In support, work by Barcellini, et al [11] and Lecouffe-Desprets, et al have suggested that thrombosis in AIHA is associated with more severe anemia at the time of AIHA onset and at the time of the thrombotic event [19,20].…”

Section: Introductionsupporting

confidence: 52%

“…beta-thalassemia, paroxysmal nocturnal hemoglobinuria, sickle cell disease) [30]. Several mechanisms for thrombosis during haemolysis have been proposed, including the abnormal exposure of phosphatidylserine following red blood cell destruction, which may make erythrocytes more adhesive and promote activation of coagulation pathways [18,30]. Others have suggested that haemolysis leads to nitric oxide depletion by free plasma hemoglobin, as well as abnormal erythrocyte-endothelium interactions that lead to elevated levels of tissue factor on vessel endothelium [30,31].…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Hemolytic Anemia Confers an Independent Risk Factor for Thrombosis: Retrospective Cohort Study Using the “STRIDE” Database

Kummar¹

2017

Ann Hematol Oncol

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“…In all cases, the thrombotic manifestation occurred with active wAIHA, either at disease onset or during relapse. The link between AIHA and venous thrombosis has been reported since 1950 and the first series of AIHA and also in the setting of other inherited [20,21] but also acquired causes of hemolytic anemia such a paroxysmal nocturnal hemoglobinuria [22]. Patients with active wAIHA with a lupus anticoagulant [23], or those undergoing splenectomy [24] should be considered at high risk of thrombosis as 3 of 9 patients who underwent laparoscopic splenectomy experienced postoperative thrombosis of the portal vein system.…”

Section: Discussionmentioning

confidence: 99%

Characteristics and outcome of warm autoimmune hemolytic anemia in adults: New insights based on a single‐center experience with 60 patients

et al. 2014

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Warm autoimmune hemolytic anemia (wAIHA) is a rare autoimmune disease with poorly known natural history and management remaining mainly empirical. To better describe the characteristics and outcome of wAIHA in adults, we performed a single-center cohort study of patients diagnosed with wAIIHA from 2001 to 2012 in our center. Sixty patients (50% women) were included, the mean age at the time of wAIHA onset was 54 6 23 years. wAIHA was considered "primary" for 21 patients (35%) and was associated with an underlying disorder in 39 (65%), including mainly lymphoproliferative disorders and systemic lupus. All patients but two needed treatment and received corticosteroids, with an overall initial response rate of 87%. However, 63% of the patients were corticosteroid-dependent and 56% required at least one second-line treatment including mainly rituximab (n 5 19). At the time of analysis, after a mean follow-up of 46 months, 28 patients (47%) were in remission and off treatment and 5 (8%) had died. The presence of an underlying lymphoproliferative disorder was associated with reduced response to corticosteroids and increased need for second-line therapy. In conclusion, in the last decade and compared to a previous series from our center, the rate of secondary wAIHA has increased and the use of rituximab has emerged as the preferred second-line treatment and corticosteroid-sparing strategy; the overall mortality has significantly decreased (8 vs. 18%).Am. J. Hematol. 89:E150-E155,

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Hypercoagulability and thrombotic complications in hemolytic anemias

Cited by 150 publications

References 41 publications

Thrombin generation reveals high procoagulant potential in the plasma of sickle cell disease children

Thrombin generation reveals high procoagulant potential in the plasma of sickle cell disease children

Autoimmune Hemolytic Anemia Confers an Independent Risk Factor for Thrombosis: Retrospective Cohort Study Using the “STRIDE” Database

Characteristics and outcome of warm autoimmune hemolytic anemia in adults: New insights based on a single‐center experience with 60 patients

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