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35 papers 25 to 100 followers
Chad S Kessler, Bilal A Khan, Katie Lai-Miller
BACKGROUND: Thrombotic thrombocytopenic purpura is a hematological emergency and diagnostic challenge. The critical determinant of outcome is timely diagnosis and treatment. OBJECTIVES: Describe the pathophysiology, presentation, diagnosis, and treatment of thrombotic thrombocytopenic purpura. DISCUSSION: Thrombotic thrombocytopenic purpura has a varied presentation and a tendency to mimic several disorders. However, it may be at least provisionally diagnosed in the patient with thrombocytopenia and microangiopathic hemolytic anemia without alternate cause...
September 2012: Journal of Emergency Medicine
Guillermo Montalban-Bravo, Guillermo Garcia-Manero
DISEASE OVERVIEW: The myelodysplastic syndromes (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older males and in individuals with prior exposure to cytotoxic therapy. DIAGNOSIS: Diagnosis of MDS is based on morphological evidence of dysplasia upon visual examination of a bone marrow aspirate and biopsy. Information obtained from additional studies such as karyotype, flow cytometry or molecular genetics is usually complementary and may help refine diagnosis...
January 2018: American Journal of Hematology
Meaghan E Colling, Pavan K Bendapudi
Purpura fulminans (PF) is a highly thrombotic subtype of disseminated intravascular coagulation that can accompany severe bacterial, and more rarely, viral infections. PF is associated with an extremely high mortality rate, and patients often die of overwhelming multisystemic thrombosis rather than septic shock. Survivors typically experience amputation of involved extremities and significant scarring in affected areas. Despite the devastating clinical course associated with this hemostatic complication of infection, the mechanism of PF remains poorly understood...
October 16, 2017: Transfusion Medicine Reviews
Kuixing Zhang, Yuxin Lu, Kevin T Harley, Minh-Ha Tran
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury...
June 1, 2017: Hematology Reports
Jeffrey Glassberg
Sickle cell disease (SCD) is the most common genetic disease in the US, affecting approximately 100,000 individuals. In SCD, genetically mutated hemoglobin (HbS) forms rigid polymers when deoxygenated, giving red blood cells a characteristic sickled shape. Increased blood viscosity and cell adhesion produce intermittent vaso-occlusion. The vaso-occlusive phenotype of SCD, which is marked by higher hemoglobin, manifests with frequent painful crises and is associated with a higher risk for developing acute chest syndrome...
August 2011: Emergency Medicine Practice
Giuseppe Lippi, Massimo Franchini, Martina Montagnana, Emmanuel J Favaloro
Hemostasis is traditionally defined as a physiological response to blood vessel injury and bleeding, which entails a co-ordinated process involving the blood vessel, platelets, and blood clotting proteins (i.e. coagulation factors). Hemostasis can be divided into primary and secondary components. The former rapidly initiates after endothelial damage and is characterized by vascular contraction, platelet adhesion, and formation of a soft aggregate plug. The latter is initiated following the release of tissue factor and involves a complex sequence of events known as the blood coagulation cascade, encompassing serial steps where each coagulation factor activates another in a chain reaction that culminates in the conversion of fibrinogen to fibrin...
August 2012: Annals of Medicine
Dominick J Angiolillo
No abstract text is available yet for this article.
October 14, 2017: Lancet
Dipesh Uprety, Aurangzeb Baber, Maria Foy
This article reports a rare case of the use of low-dose ketamine infusion as an adjuvant to opioids to treat pain in sickle cell disease. A 31-year-old African-American male with history of sickle cell disease presented to the emergency department with complaints of chest tightness, multiple joint pain, and headache for 1 week. His vital signs and physical examination were unremarkable. His admission lab included hemoglobin of 8.4 g/dl, reticulocyte count of 16.3%, bilirubin of 1.7 mg/dl, and LDH of 1,267 U/l...
May 2014: Annals of Hematology
Eleana Gkamprela, Melanie Deutsch, Dimitrios Pectasides
Chronic liver disease is accompanied by multiple hematological abnormalities. Iron deficiency anemia is a frequent complication of advanced liver disease. The etiology is multifactorial, mostly due to chronic hemorrhage into the gastrointestinal tract. The diagnosis of iron deficiency anemia is very challenging, as simple laboratory methods, including serum iron, ferritin, transferrin saturation (Tsat), and mean corpuscular volume are affected by the liver disease itself or the cause of the disease, resulting in difficulty in the interpretation of the results...
2017: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
T Terwilliger, M Abdul-Hay
Acute lymphoblastic leukemia (ALL) is the second most common acute leukemia in adults, with an incidence of over 6500 cases per year in the United States alone. The hallmark of ALL is chromosomal abnormalities and genetic alterations involved in differentiation and proliferation of lymphoid precursor cells. In adults, 75% of cases develop from precursors of the B-cell lineage, with the remainder of cases consisting of malignant T-cell precursors. Traditionally, risk stratification has been based on clinical factors such age, white blood cell count and response to chemotherapy; however, the identification of recurrent genetic alterations has helped refine individual prognosis and guide management...
June 30, 2017: Blood Cancer Journal
Kelsey Preston, Sarah Harm, Nathan Dreyfus, Nuria Villalba, Kalev Freeman
No abstract text is available yet for this article.
August 2017: Shock
Keneisha Bailey, Jagila Wesley, Adebayo Adeyinka, Louisdon Pierre
Fat embolism syndrome (FES) has been described in the literature as a rare complication of sickle cell disease (SCD). A review article published in 2005 reported 24 cases of FES associated with SCD. In many cases, a definitive diagnosis of FES in SCD is made on autopsy because of the lack of early recognition and the paucity of sensitive and specific testing for this syndrome. Patients with FES usually have a fulminant, rapidly deteriorating clinical course with mortality occurring within the first 24 hours...
January 1, 2017: Journal of Intensive Care Medicine
Lena M Napolitano
Anemia is common in the intensive care unit (ICU), resulting in frequent administration of red blood cell (RBC) transfusions. Significant advances have been made in understanding the pathophysiology of anemia in the ICU, which is anemia of inflammation. This anemia is related to high hepcidin concentrations resulting in iron-restricted erythropoiesis, and decreased erythropoietin concentrations. A new hormone (erythroferrone) has been identified, which mediates hepcidin suppression to allow increased iron absorption and mobilization from iron stores...
April 2017: Critical Care Clinics
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving protease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies, but rarely, it is inherited via mutations of the ADAMTS13 gene...
May 25, 2017: Blood
Smita Padhi, Sophia Kemmis-Betty, Sharangini Rajesh, Jennifer Hill, Michael F Murphy
No abstract text is available yet for this article.
November 18, 2015: BMJ: British Medical Journal
Sylvain Audia, Matthieu Mahévas, Maxime Samson, Bertrand Godeau, Bernard Bonnotte
Immune thrombocytopenia (ITP) is a rare autoimmune disease due to an abnormal T cell response, notably supported by splenic T follicular helper cells, that stimulates the proliferation and differentiation of autoreactive B cells. The antiplatelet autoantibodies they produce facilitate platelet phagocytosis by macrophages, essentially in the spleen. Macrophages contribute to the perpetuation of the auto-immune response as the main antigen-presenting cell during ITP. CD8+ T cells also participate to thrombocytopenia by increasing platelet apoptosis...
June 2017: Autoimmunity Reviews
William B Mitchell, James B Bussel
Thrombopoietic agents have created a paradigm shift in the management of chronic or poorly responsive immune thrombocytopenia (ITP). There are ample randomized, placebo-controlled trial data, as well as long-term data gathered for more than 5 years; short-term efficacy and safety are well documented and long-term efficacy and safety data are emerging. The purpose of this review will be to focus critically on what we know or do not know at this point about these agents. We review the evolution, efficacy and clinical use, side effects, special populations, and off-target effects of the thrombopoietic agents...
January 2015: Seminars in Hematology
Phil Hui, Deborah J Cook, Wendy Lim, Graeme A Fraser, Donald M Arnold
BACKGROUND: The epidemiology of thrombocytopenia in critically ill patients has not been well characterized. The objective of this study was to systematically review the prevalence, incidence, and consequences of, and risk factors for, thrombocytopenia among critically ill patients. METHODS: We searched MEDLINE, EMBASE, the Cochrane Registry for controlled trials (until May 2010), and the Online Computer Library, as well as bibliographies of relevant studies, to identify investigations designed to examine the frequency, risk factors, and/or outcomes associated with thrombocytopenia among patients admitted to the ICU...
February 2011: Chest
Benjamin S Salter, Menachem M Weiner, Muoi A Trinh, Joshua Heller, Adam S Evans, David H Adams, Gregory W Fischer
Heparin-induced thrombocytopenia is a profoundly dangerous, potentially lethal, immunologically mediated adverse drug reaction to unfractionated heparin or, less commonly, to low-molecular weight heparin. In this comprehensive review, the authors highlight heparin-induced thrombocytopenia's risk factors, clinical presentation, pathophysiology, diagnostic principles, and treatment. The authors place special emphasis on the management of patients requiring procedures using cardiopulmonary bypass or interventions in the catheterization laboratory...
May 31, 2016: Journal of the American College of Cardiology
Radhika Dasararaju, Nirupama Singh, Amitkumar Mehta
Heparin induced thrombocytopenia (HIT) is a serious, potentially life and limb threatening immune adverse reaction to heparin. IgG antibodies against platelet factor 4 and heparin multimer complexes activate platelets to create a prothrombotic state. ELISA based immunoassay to detect these antibodies is sensitive while serotonin release assay is highly specific but is not widely available. 4T score is a simple score to calculate pre-test probability of HIT. Score < 3 is highly specific to exclude the diagnosis...
August 2013: Expert Review of Hematology
2017-06-02 23:16:48
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