Sickle Cell Disease: A Paradigm for Venous Thrombosis Pathophysiology

Lizarralde-Iragorri, Maria A.; Shet, Arun S.

doi:10.3390/ijms21155279

Cited by 31 publications

(25 citation statements)

References 133 publications

(184 reference statements)

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“…The vascular damage induced by ROS is worsened by NO reaction with superoxide, that forms peroxynitrite, a highly harmful molecule for the endothelium [ 49 ]. The resulting inflammation and hypoxia further increase tissue factor expression and the consequent activation of the coagulation cascade [ 50 ]. One of the principal consequences of this inflammatory activation caused by free heme is the release of neutrophil extracellular traps (NETs), mesh-works of chromatin fibers released by neutrophils, which favor coagulation by serving as a scaffold for the activation of platelets and coagulation factors [ 51 ].…”

Section: Pathophysiology Of Thrombosis In Immune Mediated Hemolysismentioning

confidence: 99%

Thrombotic Complications in Patients with Immune-Mediated Hemolysis

Capecchi

Ciavarella

Artoni

et al. 2021

JCM

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Autoimmune hemolytic anemias are rare and heterogeneous disorders characterized by hemolysis, which is a well-recognized risk factor for thrombosis. The most common immune-mediated anemias are represented by autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria, both associated with a high rate of thrombosis. Multiple pathophysiological mechanisms for thrombosis have been proposed, involving hemolysis itself and additional effects of the immune system. Despite the increasing awareness of the thrombotic risk in these conditions, evidence-based guidance on prevention and management of thrombotic events is lacking. We herein report available evidence on epidemiological data on thrombosis and thrombophilia in immune-mediated hemolysis, together with possible underlying pathophysiological mechanisms. In addition, we summarize current recommendations for treatment of thrombosis in immune-mediated hemolysis. In particular, we address the issue of thrombotic complications treatment and prophylaxis by proposing a therapeutic algorithm, focusing on specific situations such as splenectomy and pregnancy.

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Section: Pathophysiology Of Thrombosis In Immune Mediated Hemolysismentioning

confidence: 99%

Thrombotic Complications in Patients with Immune-Mediated Hemolysis

Capecchi

Ciavarella

Artoni

et al. 2021

JCM

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“…Virchow’s triad of hypercoagulability, endothelial dysfunction, and stasis, the underlying pathophysiology of venous thrombosis, is usually present in SCD patients, leading to an increased risk of thromboembolic events [ 5 ]. The average age at which venous thrombosis is diagnosed in the general population is 65 years, while the average age for high-risk thrombophilia is 30 years, which is comparable to the average age at which venous thrombosis of SCD is diagnosed [ 4 ]. A cross-sectional study of 404 patients at the Johns Hopkins Sickle Cell Center reported a median age of venous thrombosis diagnosis in SCD of 29.9 years, with 25% of the study population reporting a history of venous thrombosis [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

“…There is an increased risk of venous thrombosis and pulmonary embolism in SCD patients with associated significant mortality [ 4 ]. A high index of suspicion is important for early diagnosis, and prompt intervention is warranted for optimal outcomes.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Embolism and Chronic Superior Vena Cava Occlusion Complicating Central Line-Associated Venous Thromboembolism in a Sickle Cell Disease Patient

Brgdar¹,

Ojo²,

Rougui³

et al. 2022

Cureus

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Sickle cell disease (SCD), the most common genetic disorder globally, is often associated with an increased risk of venous thromboembolic events (VTE). Many of these patients have central lines placed for the purposes of repeated medication administration, blood transfusions, and blood draw, further increasing the risk of VTE. Given the non-specific presentation of VTE and pulmonary embolism, as well as the risk of mortality if interventions are delayed, a high index of suspicion is required for early diagnosis of the condition. We report the case of a 35-year-old woman with SCD and a port-a-cath in place who presented with extensive upper extremity and intrathoracic VTE with associated pulmonary embolism and chronic superior vena cava (SVC) occlusion. We also discuss the peculiarities of the clinical manifestations and management of VTE and pulmonary embolism in the setting of SCD based on the evidence from existing literature.

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“…RBCs expressing the HOD antigen, transfused into animals with SCD (Hgb SS), did not result in an increase in responsiveness or in the magnitude of responsiveness compared with those transfused into animals with Hgb AS or Hgb AA ( 67 ). Murine models of SCD, summarized by Lizarralde-Iragorri and Shet ( 68 ), are just that; as such, it is not entirely clear how to interpret these data in the context of human SCD.…”

Section: Inflammation and Rbc Alloantibody Induction In Murine Modelsmentioning

confidence: 99%

Red blood cell alloimmunization and sickle cell disease: a narrative review on antibody induction

Hendrickson¹

2020

Ann Blood

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The high prevalence of red blood cell (RBC) alloantibodies in people with sickle cell disease (SCD) cannot be debated. Why people with SCD are so likely to form RBC alloantibodies, however, remains poorly understood. Over the past decade, a better understanding of non-ABO blood group antigen variants has emerged; RH genetic diversity and the role this diversity plays in RBC alloimmunization is discussed elsewhere. Outside of antigen variants, the immune systems of people with SCD are known to be different than those of people without SCD. Some of these differences are due to effects of free heme, whereas others are impacted by hyposplenism. Descriptive studies of differences in white blood cell (WBC) subsets, platelet counts and function, and complement activation between people with SCD and race-matched controls exist. Studies comparing the immune systems of alloimmunized people with SCD to non-alloimmunized people with SCD to race-matched controls without SCD have uncovered differences in T-cell subsets, monocytes, Fcγ receptor polymorphisms, and responses to free heme. Studies in murine models have documented the role that recipient inflammation plays in RBC alloantibody formation, with human studies reporting a similar association. Murine studies have also reported the importance of type 1 interferon (IFNα/β), known to play a pivotal role in autoimmunity, in RBC alloantibody formation. The goal of this manuscript is to review existing data on factors influencing RBC alloantibody induction in people with SCD with a focus on inflammation and other immune system considerations, from the bench to the bedside.

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Sickle Cell Disease: A Paradigm for Venous Thrombosis Pathophysiology

Cited by 31 publications

References 133 publications

Thrombotic Complications in Patients with Immune-Mediated Hemolysis

Thrombotic Complications in Patients with Immune-Mediated Hemolysis

Pulmonary Embolism and Chronic Superior Vena Cava Occlusion Complicating Central Line-Associated Venous Thromboembolism in a Sickle Cell Disease Patient

Red blood cell alloimmunization and sickle cell disease: a narrative review on antibody induction

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