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Sickle cell disease thrombosis

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
V van Hamel Parsons, K Gardner, R Patel, S L Thein
Venous thromboembolism (VTE) is a recognised complication of sickle cell disease (SCD), long considered to be a hypercoagulable state. While there is a good understanding of arterial thrombosis in SCD, the nature of VTE in SCD is less well-characterised. In this retrospective cohort study, we found that the incidence of VTE in our patient cohort was higher than in the non-SCD black population; patients of all SCD genotypes with VTE had significantly elevated steady-state platelet counts compared to those without...
January 2016: Annals of Hematology
Yimo Lin, Dominic Harris, I-Wen Pan, Thomas G Luerssen, Sandi Lam
INTRODUCTION: Moyamoya is a rare disease, and thus there have been few published studies with sufficient power for outcomes analyses of bypass surgery performed in the modern era. METHODS: This Kids Inpatient Database (KID) was queried for hospitalizations of pediatric patients with Moyamoya disease undergoing extracranial-intracranial vascular bypass surgery in the years 2003, 2006, and 2009. Type of bypass (direct vs indirect) was not distinguishable in coding...
August 2015: Neurosurgery
Elisabetta Radin, Cristina Izzo, Marco Quaglia, Guido Merlotti, Angelo Nappo, Michele Battista, Gabriele Guglielmetti, Piero Stratta
BACKGROUND: Often the reduced contrast enhancement on CT renal imaging is radiologically interpreted as acute pyelonephritis (PNA), but it is the task of the clinician to assess a possible differential diagnosis such as a renal infarct and look for a cause. METHODS: In our experience (2010-2013), we hospitalized 51 patients with radiological imaging consistent with acute pyelonephritis in native kidneys. However, three of these cases result, after a second look, to be ischemic lesions, only sometimes complicated by over-infections (Tabella 1)...
March 2015: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
Nicolas de Prost, Myriam Sasanelli, Jean-François Deux, Anoosha Habibi, Keyvan Razazi, Frédéric Galactéros, Michel Meignan, Bernard Maître, Christian Brun-Buisson, Emmanuel Itti, Armand Mekontso Dessap
The acute chest syndrome (ACS) is the main cause of mortality among adult patients with sickle cell disease (SCD). Its pathophysiology is still unclear. Using positron emission tomography (PET) with F-fluorodeoxyglucose [18F-fluorodeoxyglucose (F-FDG)], we explored the relationship between regional lung density and lung metabolism, as a reflection of lung neutrophilic infiltration during ACS.Patients were prospectively enrolled in a single-center study. Dual modality chest PET/computed tomography (CT) scans were performed, with F-FDG emission scans for quantification of regional F-FDG uptake and CT scans with radiocontrast agent to check for pulmonary artery thrombosis...
May 2015: Medicine (Baltimore)
Tiago de Oliveira Boechat, Emilia Matos do Nascimento, Clarisse Lopes de Castro Lobo, Samir K Ballas
BACKGROUND: Deep venous thrombosis (DVT) is rare in children compared to adults. Its incidence and risk factors in children are not well known. This study determined these aspects of DVT in children with sickle cell disease (SCD). PROCEDURE: A retrospective, observational and descriptive study was performed. Patients born between October 2000 and October 2012 with SCD and registered in HEMORIO, including those who died in HEMORIO, were included in this study. Patients whose medical records were inaccessible, who died in institutions other than HEMORIO, who died with implanted deep venous catheters, and those who were not monitored in HEMORIO for a period of 1 year or more were excluded from the study...
May 2015: Pediatric Blood & Cancer
Ali Dahhan
Coronary artery ectasia (CAE) or aneurysm is usually defined as dilation ≥1.5-fold the normal vessel diameter. It has an incidence of 1.4-5.3% and is associated with a wide variety of etiologies-mainly congenital, atherosclerotic, and inflammatory ones. CAE is very common in sickle cell disease, and possibly sickle cell trait, with an incidence of 17.7%. It is likely related to the inflammatory process associated with hemoglobin S. Prognosis depends mainly on the underlying etiology. Atherosclerotic CAE does not carry additional risks compared to atherosclerotic coronary artery disease (ACAD) without ectasia...
April 2015: Cardiovascular Therapeutics
Jennifer Davila, Deepa Manwani, Ljiljana Vasovic, Mauro Avanzi, Joan Uehlinger, Karen Ireland, W Beau Mitchell
The severe pain, ischemia and organ damage that characterizes sickle cell disease (SCD) is caused by vaso-occlusion, which is the blockage of blood vessels by heterotypic aggregates of sickled erythrocytes and other cells. Vaso-occlusion is also a vasculopathy involving endothelial cell dysfunction, leukocyte activation, platelet activation and chronic inflammation resulting in the multiple adhesive interactions between cellular elements. Since platelets mediate inflammation as well as thrombosis via release of pro- and anti-inflammatory molecules, we hypothesized that platelets may play an active inflammatory role in SCD by secreting increased amounts of cytokines...
2015: Platelets
Awa Ot Fall, Valérie Proulle, Abibatou Sall, Alassane Mbaye, Pape Samba Ba, Maboury Diao, Moussa Seck, Macoura Gadji, Sara B Gning, Saliou Diop, Tandakha Nd Dièye, Blaise Félix Faye, Doudou Thiam, Marie Dreyfus
Little is known about the biological, epidemiological, and clinical risk factors for thrombosis and venous thromboembolism (VTE) among Black Africans. We undertook a study of the prevalence of VTE risk factors for thrombosis in a Senegalese population. A three-year cross-sectional and case-control study involving 105 cases and 200 controls was conducted in various hospitals in Dakar (Senegal). Our results demonstrate that oral contraception, immobilization by casts, surgery, and blood group were significantly associated with VTE occurrence...
2014: Clinical Medicine Insights. Blood Disorders
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