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Sickle cell disease thrombosis

Laura Beth Mann Dosier, Vikram J Premkumar, Hongmei Zhu, Izzet Akosman, Michael F Wempe, Timothy J McMahon
The system L neutral amino acid transporter (LAT; LAT1, LAT2, LAT3, or LAT4) has multiple functions in human biology, including the cellular import of S-nitrosothiols (SNOs), biologically active derivatives of nitric oxide (NO). SNO formation by haemoglobin within red blood cells (RBC) has been studied, but the conduit whereby a SNO leaves the RBC remains unidentified. Here we hypothesised that SNO export by RBCs may also depend on LAT activity, and investigated the role of RBC LAT in modulating SNO-sensitive RBC-endothelial cell (EC) adhesion...
April 6, 2017: Thrombosis and Haemostasis
Renita E Horton
Sickle Cell Disease (SCD) stems from a mutation in the beta globin gene. Upon deoxygenation, hemoglobin polymerizes and triggers red blood cell remodeling. This phenomenon is central to SCD pathogenesis as individuals suffering from the disease are plagued by painful vaso-occlusive crises episodes. These episodes are the result of a combination of processes including inflammation, thrombosis, and blood cell adhesion to the vascular wall which leads to blockages within the vasculature termed vaso-occlusions...
April 4, 2017: Microcirculation: the Official Journal of the Microcirculatory Society, Inc
Ann Brunson, Amy Lei, Aaron S Rosenberg, Richard H White, Theresa Keegan, Ted Wun
Previous reports show increased incidence of venous thromboembolism [VTE, deep-vein thrombosis (DVT) and pulmonary embolus (PE)] in sickle cell disease (SCD) patients but did not account for frequency of hospitalization. We determined the incidence of VTE in a SCD cohort versus matched controls. For SCD patients, risk factors for incident VTE, recurrence and the impact on mortality were also determined. Among 6237 patients with SCD, 696 patients (11·2%) developed incident-VTE: 358 (51·6%) had PE (±DVT); 179 (25·7%) had lower-extremity DVT only and 158 (22·7%) had upper-extremity DVT...
April 3, 2017: British Journal of Haematology
Michel W J Smeets, Ruben Bierings, Henriet Meems, Frederik P J Mul, Dirk Geerts, Alexander P J Vlaar, Jan Voorberg, Peter L Hordijk
Adhesion of erythrocytes to endothelial cells lining the vascular wall can cause vaso-occlusive events that impair blood flow which in turn may result in ischemia and tissue damage. Adhesion of erythrocytes to vascular endothelial cells has been described in multiple hemolytic disorders, especially in sickle cell disease, but the adhesion of normal erythrocytes to endothelial cells has hardly been described. It was shown that calcium-loaded erythrocytes can adhere to endothelial cells. Because sickle erythrocyte adhesion to ECs can be enhanced by ultra-large von Willebrand factor multimers, we investigated whether calcium loading of erythrocytes could promote binding to endothelial cells via ultra-large von Willebrand factor multimers...
2017: PloS One
Gowtham K Annarapu, Rashi Singhal, Avinash Gupta, Sheetal Chawla, Harish Batra, Tulika Seth, Prasenjit Guchhait
Intravascular hemolysis increases the risk of thrombosis in hemolytic disorders. Our previous study showed that the binding of adult hemoglobin (HbA) to glycoprotein (GP) 1bα induced the activation of platelets. The elevated plasma Hb or platelet surface bound Hb positively correlated with platelet activation in patients with paroxysmal nocturnal hemoglobinuria (PNH). Furthermore, this study shows that the sickle Hb [HbS, occurs due to single nucleotide polymorphism at A>T of β-globin gene of Hb and causes sickle cell disease (SCD)] also bound to GP1bα and activated platelets in a concentration-dependent manner...
2016: PloS One
Natália S Carvalho, Josefina Pellegrini Braga, Márcia Barbieri, Maria R Torloni, Maria S Figueiredo, Cristina A F Guazzelli
This was a cross-sectional study of sexually active women with sickle-cell disease (SCD) managed at São Paulo Federal University during a one-year period. A total of 54 women were included. Mean age was 32.0 (11.2, standard deviation) years and almost 95% were black or of mixed racial ancestry. Almost 80% reported a history of multiple blood transfusions, 50% had kidney disease and 17% had a history of thrombosis. Over 80% of them had used some form of contraception, mostly combined hormonal contraceptive (52%) or progestin-only contraceptives (46%)...
December 7, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
Ted Wun, Ann Brunson
Activation of the hemostatic system occurs in patients with sickle cell disease. The extent to which this activation contributes to sickle cell pathophysiology is uncertain. Clinical trials of anticoagulants or platelet inhibitors have demonstrated the ability to decrease biomarkers of hemostatic activation, but this has generally not resulted in improvement in clinically relevant outcomes. Venous thromboembolism (VTE: deep venous thrombosis and pulmonary embolism) has been until recently an underappreciated complication of sickle cell disease, with incident event and recurrence rates consistent with a strong thrombophilia...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
Marianne Delville, Sandra Manceau, Nassim Ait Abdallah, Jan Stolba, Sameh Awad, Thibaud Damy, Barnabas Gellen, Laurent Sabbah, Karima Debbache, Vincent Audard, Jean-Louis Beaumont, Cécile Arnaud, Christelle Chantalat-Auger, Françoise Driss, François Lefrère, Marina Cavazzana, Gilbert Franco, Frederic Galacteros, Jean-Antoine Ribeil, Justine Gellen-Dautremer
Erythrocytapheresis (ER) can improve outcome in patients with sickle cell disease (SCD). A good vascular access is required but frequently it can be difficult to obtain for sickle cell patients. Arterio-venous fistulas (AVFs) have been suggested for ER in SCD supported by limited evidence. We report the largest cohort of ER performed with AVFs from three French SCD reference centers. Data of SCD patients undergoing ER with AVFs in the French SCD reference center were retrospectively collected. The inclusion criteria were: SS or Sβ-Thalassemia and AVF surgery for ER...
February 2017: American Journal of Hematology
Daniel R Couriel, Holly Ricker, Mary Steinbach, Catherine J Lee
Neurologic manifestations are common in blood diseases, and they can be caused by the hematologic disorder or its treatment. This article discusses hematologic diseases in adult patients, and categorizes them into benign and malignant conditions. The more common benign hematologic diseases associated with neurologic manifestations include anemias, particularly caused by B12 deficiency and sickle cell disease, and a variety of disorders of hemostasis causing bleeding or thrombosis, including thrombotic microangiopathy...
August 2016: Hematology/oncology Clinics of North America
Andrei S Purysko, Antonio C Westphalen, Erick M Remer, Christopher P Coppa, Hilton M Leão Filho, Brian R Herts
The kidneys and perinephric tissues can be affected by a variety of hematologic disorders, which usually occur in the setting of multisystem involvement. In many of these disorders, imaging is used to evaluate the extent of disease, guide biopsy, and/or monitor disease activity and patient response to therapy. Lymphoma, leukemia, and multiple myeloma commonly manifest as multiple parenchymal or perinephric lesions. Erdheim-Chester disease and Rosai-Dorfman disease, rare forms of multisystemic histiocytosis, are often identified as perinephric and periureteral masses...
July 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Takana Yamakawa Hayashi, Izuru Matsuda, Kazuchika Hagiwara, Tomoko Takayanagi, Akifumi Hagiwara
We report a case of splenic infarction in a patient with sickle cell traits (SCT), focusing on the computed tomography (CT) findings. The patient was an African-American man in his twenties with no past medical history who experienced sudden left upper quadrant pain while climbing a mountain (over 3000 m above sea level). Dynamic contrast-enhanced CT revealed massive non-segmental splenic infarction accompanied with nodule-like preserved splenic tissue. The region of splenic infarction did not coincide with the arterial vascular territory and differed from the features of infarction caused by large arterial embolism...
September 2016: Abdominal Radiology
Rebecca H Stone, Adam P Bress, Edith A Nutescu, Nancy L Shapiro
BACKGROUND: Upper-extremity deep-vein thrombosis (UEDVT) causes significant morbidity and mortality and is not well characterized in the existing literature, particularly in underrepresented minorities such as African Americans. OBJECTIVE: To describe the characteristics of a cohort of patients with UEDVT seen at an urban academic medical center. METHODS: This was a retrospective cohort study among patients with a confirmed UEDVT at the University of Illinois Hospital and Health Sciences System between 1996 and 2011...
August 2016: Annals of Pharmacotherapy
John Chapin, Hunter S Terry, Dorothy Kleinert, Jeffrey Laurence
OBJECTIVE: The objective of this study was to describe complement activation in hemostatic and pathologic states of coagulation and in the acquired and congenital hemolytic anemias. METHODS AND RESULTS: We review published and emerging data on the involvement of the classic, alternative and lectin-based complement pathways in coagulation and the hemolytic anemias. The alternative pathway in particular is always "on," at low levels, and is particularly sensitive to hyper-activation in a variety of physiologic and pathologic states including infection, autoimmune disorders, thrombosis and pregnancy, requiring tight control predicated on a variety of soluble and membrane bound regulatory proteins...
April 2016: Transfusion and Apheresis Science
Claudia Maximo, Sara T Olalla Saad, Eleonora Thome, Ana Maria Mach Queiroz, Clarisse Lobo, Samir K Ballas
In this study, we describe four new patients with sickle cell disease who had limb amputations. Two of the patients had sickle cell anemia [Hb S (HBB: c.20A > T) (β(S)/β(S))] with refractory leg ulcers that required amputations. The third patient had sickle cell trait with an extensive leg ulcer that was associated with epidermoid carcinoma. The fourth patient had amputations of both forearms and feet due to a misdiagnosis of dactylitis. Review of the literature showed that the indications for amputations in sickle cell disease included three distinct categories: mythical beliefs, therapeutic and malpractice...
June 2016: Hemoglobin
Brandon M Fox, Malgorzata Kasztan
Sickle cell disease (SCD) is a genetic hematologic disorder that is characterized by a variety of potentially life threatening acute and chronic complications. Currently, hydroxyurea is the only clinically approved pharmacological therapy for the treatment of SCD, and the continued prevalence of severe disease complications underscores the desperate need for the development of new therapeutic agents. Central features of the sickle cell disease milieu, including hypoxia, oxidative stress, and thrombosis, are established enhancers of endothelin-1 (ET-1) synthesis...
August 15, 2016: Life Sciences
Marzieh Nikparvar, Mohammad Reza Evazi, Tasnim Eftekhari, Farzaneh Moosavi
In patients with sickle cell disease, thrombotic microangiopathy is a rare complication. Also in sickle cell disease, intracardiac thrombus formation without structural heart diseases or atrial arrhythmias is a rare phenomenon. We herein describe a 22-year-old woman, who was a known case of sickle cell-βthalassemia, had a history of recent missed abortion, and was admitted with a vaso-occlusive crisis. The patient had manifestations of microangiopathic hemolytic anemia, including laboratory evidence of hemolytic anemia, thrombocytopenia, respiratory distress, fever, jaundice, and abnormal liver function and coagulation tests, accompanied by clot formation on the Eustachian valve of the inferior vena cava in the right atrium and also a long and worm-like thrombus in the right ventricle...
March 2016: Iranian Journal of Medical Sciences
Angeliki Vgontzas, Larry Charleston, Matthew S Robbins
Children and adolescents with sickle cell disease (SCD) have a high prevalence of recurrent headaches (24.0-43.9 %). Acute presentation with headache can be diagnostically challenging, as the clinician must consider evaluation of several potentially devastating conditions including vascular diseases (stroke, hemorrhage, venous sinus thrombosis, moyamoya, posterior reversible encephalopathy syndrome), facial and orbital bone infarcts, dental pain, and osteomyelitis. Patients with SCD and primary headache disorders may benefit from comprehensive headache treatment plans that include abortive therapy, prophylactic therapy, and non-pharmacological modalities...
March 2016: Current Pain and Headache Reports
Matthew M Heeney, Carolyn C Hoppe, Miguel R Abboud, Baba Inusa, Julie Kanter, Bernhards Ogutu, Patricia B Brown, Lori E Heath, Joseph A Jakubowski, Chunmei Zhou, Dmitry Zamoryakhin, Tsiri Agbenyega, Raffaella Colombatti, Hoda M Hassab, Videlis N Nduba, Janet N Oyieko, Nancy Robitaille, Catherine I Segbefia, David C Rees
BACKGROUND: Sickle cell anemia is an inherited blood disorder that is characterized by painful vaso-occlusive crises, for which there are few treatment options. Platelets mediate intercellular adhesion and thrombosis during vaso-occlusion in sickle cell anemia, which suggests a role for antiplatelet agents in modifying disease events. METHODS: Children and adolescents 2 through 17 years of age with sickle cell anemia were randomly assigned to receive oral prasugrel or placebo for 9 to 24 months...
February 18, 2016: New England Journal of Medicine
Elizabeth J Bell, Pamela L Lutsey, Saonli Basu, Mary Cushman, Susan R Heckbert, Donald M Lloyd-Jones, Aaron R Folsom
BACKGROUND: Greater public awareness of venous thromboembolism may be an important next step for optimizing venous thromboembolism prevention and treatment. "Lifetime risk" is an easily interpretable way of presenting risk information. Therefore, we sought to calculate the lifetime risk of venous thromboembolism (deep vein thrombosis or pulmonary embolism) using data from 2 large, prospective cohort studies: the Cardiovascular Health Study (CHS) and the Atherosclerosis Risk in Communities (ARIC) study...
March 2016: American Journal of Medicine
Mei Lin Z Bissonnette, Kammi J Henriksen, Kristie Delaney, Nicole Stankus, Anthony Chang
Sickle cell nephropathy is a common complication in patients with sickle cell hemoglobinopathies. In these disorders, polymerization of mutated hemoglobin S results in deformation of red blood cells, which can cause endothelial cell injury in the kidney that may lead to thrombus formation when severe or manifest by multilayering of the basement membranes (glomerular and/or peritubular capillaries) in milder forms of injury. As the injury progresses, the subsequent ischemia, tubular dysfunction, and glomerular scarring can result in CKD or ESRD...
May 2016: Journal of the American Society of Nephrology: JASN
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