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Dominick J Angiolillo
No abstract text is available yet for this article.
August 25, 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...
April 17, 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
Gowthami M Arepally
Heparin-induced thrombocytopenia (HIT) is an immune complication of heparin therapy caused by antibodies to complexes of platelet factor 4 (PF4) and heparin. Pathogenic antibodies to PF4/heparin bind and activate cellular FcγRIIA on platelets and monocytes to propagate a hypercoagulable state culminating in life-threatening thrombosis. It is now recognized that anti-PF4/heparin antibodies develop commonly after heparin exposure, but only a subset of sensitized patients progress to life-threatening complications of thrombocytopenia and thrombosis...
May 25, 2017: Blood
Koji Eto, Shinji Kunishima
Thrombocytopenia is defined as a status in which platelet numbers are reduced. Imbalance between the homeostatic regulation of platelet generation and destruction is 1 potential cause of thrombocytopenia. In adults, platelet generation is a 2-stage process entailing the differentiation of hematopoietic stem cells into mature megakaryocytes (MKs; known as megakaryopoiesis) and release of platelets from MKs (known as thrombopoiesis or platelet biogenesis). Until recently, information about the genetic defects responsible for congenital thrombocytopenia was only available for a few forms of the disease...
March 10, 2016: Blood
Ronald S Go, Jeffrey L Winters, Neil E Kay
Autoimmune hemolytic anemia (AIHA) is an uncommon entity that presents diagnostic, prognostic, and therapeutic dilemmas despite being a well-recognized entity for over 150 years. This is because of significant differences in the rates of hemolysis and associated diseases and because there is considerable clinical heterogeneity. In addition, there is a lack of clinical trials required to refine and update standardized and evidence-based therapeutic approaches. To aid the clinician in AIHA management, we present four vignettes that represent and highlight distinct clinical presentations with separate diagnostic and therapeutic pathways that we use in our clinical practice setting...
June 1, 2017: Blood
Sergio Cortelazzo, Andrés Ferreri, Dieter Hoelzer, Maurilio Ponzoni
Lymphoblastic lymphoma (LBL) is a neoplasm of immature B cells committed to the B-(B-LBL) or T-cell lineage (T-LBL) that accounts for approximately 2% of all lymphomas. Although histological features are usually sufficient to distinguish lymphoblastic from mature B- or T-cell neoplasms, of greater importance for diagnosis is the characterization of immunophenotype by flow cytometry. LBL occurs more commonly in children than in adults, mostly in males. A bone marrow involvement <25% (or 20% according to WHO) formally distinguishes LBL from ALL...
May 2017: Critical Reviews in Oncology/hematology
Emma Lefrançais, Guadalupe Ortiz-Muñoz, Axelle Caudrillier, Beñat Mallavia, Fengchun Liu, David M Sayah, Emily E Thornton, Mark B Headley, Tovo David, Shaun R Coughlin, Matthew F Krummel, Andrew D Leavitt, Emmanuelle Passegué, Mark R Looney
Platelets are critical for haemostasis, thrombosis, and inflammatory responses, but the events that lead to mature platelet production remain incompletely understood. The bone marrow has been proposed to be a major site of platelet production, although there is indirect evidence that the lungs might also contribute to platelet biogenesis. Here, by directly imaging the lung microcirculation in mice, we show that a large number of megakaryocytes circulate through the lungs, where they dynamically release platelets...
April 6, 2017: Nature
Dachuan Zhang, Chunliang Xu, Deepa Manwani, Paul S Frenette
Sickle cell disease (SCD) is a severe genetic blood disorder characterized by hemolytic anemia, episodic vaso-occlusion, and progressive organ damage. Current management of the disease remains symptomatic or preventative. Specific treatment targeting major complications such as vaso-occlusion is still lacking. Recent studies have identified various cellular and molecular factors that contribute to the pathophysiology of SCD. Here, we review the role of these elements and discuss the opportunities for therapeutic intervention...
February 18, 2016: Blood
2017-05-01 14:58:58
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