DOCSLIB.ORG

Coagulopathies Evangelina Berrios- Colon, Pharmd, MPH, BCPS, CACP • Julie Anne Billedo, Pharmd, BCACP

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Coagulopathies Evangelina Berrios- Colon, Pharmd, MPH, BCPS, CACP • Julie Anne Billedo, Pharmd, BCACP CHAPTER33 Coagulopathies Evangelina Berrios- Colon, PharmD, MPH, BCPS, CACP • Julie Anne Billedo, PharmD, BCACP Coagulopathies include hemorrhage, thrombosis, and Activated protein C ( APC) inhibition is catalyzed by protein embolism, and represent common clinical manifestations of S, another vitamin K–dependent plasma protein, and also hematological disease. Normally, bleeding is controlled by requires the presence of platelet phospholipid and calcium. a fi brin clot formation, which results from the interaction Antithrombin III (AT III) primarily inhibits the activity of of platelets, plasma proteins, and the vessel wall. The fi brin thrombin and Factor X by binding to the factors and block- clot is ultimately dissolved through fi brinolysis. A derange- ing their activity. This inhibition is greatly enhanced by hep- ment of any of these components may result in a bleeding arin. Loss of function and/or decreased concentrations of or thrombotic disorder. In this chapter, individual disease these proteins result in uninhibited coagulation and hence a states are examined under the broad headings of coagulation predisposition to spontaneous thrombosis otherwise known factor defi ciencies, disorders of platelets, mixed disorders, as a hypercoagulable state. acquired thrombophilias, and inherited thrombophilias. Fibrinolysis is a mechanism for dissolving fi brin clots. Plasmin, the activated form of plasminogen, cleaves fi brin to produce soluble fragments. Fibrinolytics, such as tissue n ANATOMY, PHYSIOLOGY, AND PATHOLOGY plasminogen activator, streptokinase, and urokinase, acti- vate plasminogen, resulting in dissolution of a fi brin clot. Coagulation is initiated after blood vessels are damaged, enabling the interaction of blood with tissue factor, a pro- n CLASSES OF BLEEDING DISORDERS tein present beneath the endothelium ( Figure 33.1). Small amounts of Factor VII present in plasma bind to tissue factor, and this tissue factor–Factor VII complex activates Vascular defects usually cause bleeding only into the skin Factor X. Activated Factor X, in the presence of Factor V, and mucous membranes. Congenital causes include Osler– activates prothrombin ( II) to thrombin ( Ila), which subse- Weber– Rendu syndrome and Ehlers– Danlos disease. quently cleaves fi brinogen to fi brin. The fi brin polymer- Acquired causes of vascular purpura include infections, izes into an insoluble gel. This is stabilized by the action of drugs, uremia, connective tissue disorders, and dysprotein- Factor XIII. This process constitutes the extrinsic pathway. emias. Treatment is directed to the primary illness. Coagulation is consolidated by the intrinsic pathway. Factor XI is activated (possibly by thrombin generated in Inherited Coagulation Disorders the extrinsic pathway), resulting in the activation of Factor IX, which then activates Factor X in the presence of Factor Hereditary bleeding disorders are extremely rare. The most VIII. Activated Factor X produces a fi brin clot, as outlined common bleeding disorders include hemophilia A, hemo- in the description of the extrinsic pathway. Decreased lev- philia B, and von Willenbrand disease ( vWD). The world- els of clotting factors may be caused by defective synthesis, wide incidence of hemophilia A is 1 case per 5,000 live male excessive use, circulating inhibitors of clotting factors, or births. Hemophilia B occurs at an even lower rate, with 1 excessive proteolysis by the fi brinolytic system. case per 50,000 live male births ( Soucie, Evatt, & Jackson, The coagulation pathway is controlled by a number 1998). vWD is the most common of the three bleeding dis- of endogenous anticoagulants. Protein C is a plasma pro- orders across all ethnic groups, affecting 1% of the popu- tein that is vitamin K dependent. It requires activation by a lation. Up to 80% of affected patients have Type I vWD complex of thrombin and thrombomodulin, an endothelial ( Zimmerman & Valentino, 2013). cell protein, to inhibit activated Factors V and VIII, thus Defi ciencies of any of the known coagulation factors inhibiting the activation of Factors IX and X, respectively. could be present from birth. Defi ciencies may be inherited © Springer Publishing Company, LLC. 508 UNIT VII: Hematologic Conditions INTRINSIC PATHWAY INTRINSIC PATHWAY (Coagulation initiated by events (Coagulation initiated by events within the bloodstream) within the bloodstream) Factor XII Factor VII (Hageman Factor) (Proconvertin) Factor XI + (Plasma Thromboplastin Antecedent) Calcium (Ca++) (Factor IV) + Ca++ + Tissue Thromboplastin Factor IX (Factor III) (Plasma Thromboplastin Component) + Ca++ + Platelet Factor 3 (PF3) Factor VIII (Antihemophilic Factor) Factor X (Stuart-Power Factor) ++ Ca PF3 FIGURE 33.1 T he coagulation cascade Factor V (Proacceletin) i s initiated by the extrinsic pathway and consolidated Factor II Thrombin by the intrinsic pathway. (Prothrombin) Coagulation is controlled by Factor I Fibrin the inhibition of Factor VIII (Fibrinogen) and Factor V by activated XII Stable clot protein C (APC), and by anti- (Fibrin Stabilizing Factor) thrombin III (AT III) inhibi- tion o f Factors II and X. or result from a spontaneous disruption in the associated moderate disease (factor level: 1%–4%) or mild hemophilia coagulation factor genes. The more common defi ciencies of (factor level: 5%–25%) are at a reduced risk of spontaneous Factors VIII and IX, as well as vWD, are discussed in this hemorrhage, but may bleed excessively after trauma or sur- chapter ( Table 33.1). gery ( Wagenman, Townsend, Mathew, & Crookston, 2009). HEMOPHILIA VON WILLEBRAND DISEASE Hemophilia is an X-linked recessive disorder associated with von Willebrand factor ( vWF) is a plasma protein that is a congenital defi ciency of Factor VIII or Factor IX. Factor required for the adhesion of platelets to sites of vascular VIII defi ciency is known as hemophilia A and Factor IX damage. In persons with von Willebrand disease, defi ciency defi ciency is known as hemophilia B. Hemophilia B was of vWF is called Type I; qualitative abnormality of vWF is fi rst diagnosed by Steven Christmas and thus is also called called Type II. vWD is an autosomally inherited hemostatic Christmas disease. Hemophilia is suspected in any male who disorder of variable severity, characterized by mucosal and has excessive bleeding after trauma, or spontaneous bleeding cutaneous bleeding, similar to patients with platelet disor- into joints or soft tissues ( Table 33.1). Patients with severe ders (see Table 33.2). Patients may have a prolonged pro- hemophilia (factor level: <1%) are at risk for spontaneous thrombin time ( PT) and activated partial thromboplastin hemarthrosis and soft-tissue bleeding. Patients who have time ( aPTT). © Springer Publishing Company, LLC. CHAPTER 33: Coagulopathies 509 TABLE 33.1 Overview of Coagulopathies BLEEDING DISORDER FACTOR FREQUENCY CLINICAL PRESENTATION LAB TESTING Hemophilia A VIII 1 in 5,000 live Eccymosis Prolonged aPTT male births Hemathrosis Decreased Factor VIII Muscle hemorrhage Decreased Factor IX Wound bleeding Hemophilia B IX 1 in 50,000 live Eccymosis Prolonged aPTT male births Hemathrosis Decreased Factor IX Muscle hemorrhage Wound bleeding von Willebrand disease vWF 1% Minor bleeding Decreased von Willebrand factor- Epistaxsis antigen (vWF:Ag), Factor VIII Increased postoperative bleeding CLOTTING DISORDER FACTOR FREQUENCY CLINICAL PRESENTATION LAB TESTING Factor V Leiden V 5% Thromboembolism Positive genotypic assay APC resistance Prothrombin G20210A II 3% Thromboembolism Positive genotypic assay Protein C deficiency Protein C <1% Thromboembolism Decreased protein C activity Protein S deficiency Protein S <1% Thromboembolism Decreased protein S activity Antithrombin III ATIII <1% Thromboembolism Decreased AT III activity deficiency APC, activated protein C; aPTT, activated partial thromboplastin time; AT III, antithrombin III. Mixed Coagulation Disorders VITAMIN K DEFICIENCY Factors II, VII, IX, and X are vitamin K–dependent coagu- Mixed coagulation disorders are a group of acquired dis- lation factors that are synthesized in the liver. Vitamin K is eases. They usually involve multiple elements of the hemo- naturally synthesized by intestinal bacteria. Vitamin K is a static system and are often associated with a particular lipid-soluble vitamin, which is absorbed only in the presence disease or clinical syndrome. of bile salts. Depletion of vitamin K–dependent factors may occur in patients receiving certain antibiotics; in obstruc- tive jaundice, malabsorptive states, or hepatic parenchymal DISSEMINATED INTRAVASCULAR COAGULATION disease; and in patients receiving warfarin therapy. Patients Disseminated intravascular coagulation (DIC) can be have an elevated PT resulting in an increased international caused by the activation of either the coagulation or fibri- normalized ratio (INR). nolytic system, resulting in excessive bleeding or thrombo- sis. Conditions associated with DIC include gram-negative Platelet Disorders sepsis infections, meningococcemia, Rocky Mountain spot- ted fever, typhoid fever, obstetric complications (abruptio Platelet disorders include thrombocytopenia, which may be placentae, eclampsia, retained dead fetus), massive trauma, caused by diminished platelet production, enhanced plate- surgery, shock, and certain malignancies. let destruction, or sequestration of platelets (Table 33.2). Qualitative platelet disorders are more commonly acquired, but could be congenital. Congenital disorders affect platelet LIVER DISEASE adhesion,
Load more

Recommended publications
  • Factor XIII and Fibrin Clot Properties in Acute Venous Thromboembolism
    International Journal of Molecular Sciences Review Factor XIII and Fibrin Clot Properties in Acute Venous Thromboembolism Michał Z ˛abczyk 1,2 , Joanna Natorska 1,2 and Anetta Undas 1,2,* 1 John Paul II Hospital, 31-202 Kraków, Poland; [email protected] (M.Z.); [email protected] (J.N.) 2 Institute of Cardiology, Jagiellonian University Medical College, 31-202 Kraków, Poland * Correspondence: [email protected]; Tel.: +48-12-614-30-04; Fax: +48-12-614-21-20 Abstract: Coagulation factor XIII (FXIII) is converted by thrombin into its active form, FXIIIa, which crosslinks fibrin fibers, rendering clots more stable and resistant to degradation. FXIII affects fibrin clot structure and function leading to a more prothrombotic phenotype with denser networks, characterizing patients at risk of venous thromboembolism (VTE). Mechanisms regulating FXIII activation and its impact on fibrin structure in patients with acute VTE encompassing pulmonary embolism (PE) or deep vein thrombosis (DVT) are poorly elucidated. Reduced circulating FXIII levels in acute PE were reported over 20 years ago. Similar observations indicating decreased FXIII plasma activity and antigen levels have been made in acute PE and DVT with their subsequent increase after several weeks since the index event. Plasma fibrin clot proteome analysis confirms that clot-bound FXIII amounts associated with plasma FXIII activity are decreased in acute VTE. Reduced FXIII activity has been associated with impaired clot permeability and hypofibrinolysis in acute PE. The current review presents available studies on the role of FXIII in the modulation of fibrin clot properties during acute PE or DVT and following these events.
    [Show full text]
  • Protein S Deficiency Presenting with Hemorrhage in a Term Neonate
    : Curre re nt a R C e Ayari et al., Health Care Current Reviews 2018, 6:1 h v t i l e a w DOI: 10.4172/2375-4273.1000219 e s H Health Care: Current Reviews ISSN: 2375-4273 Review Article Open Access Protein S Deficiency Presenting with Hemorrhage in a Term Neonate Fairouz Ayari*, Takoua Bensmail, Essid Latifa, Wiem Barbaria and Samia Kacem Neonatology Intensive Care Unit of the Maternity and Neonatology Center, Tunis, Tunisia Abstract Unexplained bleeding symptoms in otherwise healthy full-term usually present a diagnostic challenge for treating physicians requiring prompt and accurate laboratory investigations to ensure appropriate treatment and possibly avoid long-term morbidity. We report a case of a term neonate with severe protein S deficiency manifested by systemic hemorrhage and multiple organ failure at 9 days of age. We review how protein S influences the coagulation and the fibrinolytic pathways, discussing therapeutic approaches of neonates with purpura fulminans. Keywords: Protein S deficiency; Blood sample; Thrombophilic dis- resuscitation with 20 ml/kg bodyweight (BW) saline solution and, after order blood sampling, intravenous administration of 10 mg vitamin K, 20 ml/kg BW fresh frozen plasma, 20 ml/kg BW packed red blood cells Introduction (5 transfusion cycles), 20 mg/kg BW Phenobarbital and vasoactive Protein S (PS) is an antithrombotic plasma protein that acts mainly drugs. Cerebral ultrasound revealed intraventricular haemorrhage, as a cofactor of activated protein C (APC) anticoagulant activity in the abdominal ultrasound showed splenic hemorrhage and cardiac degradation of factor Va and activated factor VIII [1]. PS circulates in ultrasound showed a floating intracardiac thrombus.
    [Show full text]
  • WFH Treatment Guidelines 3Ed Chapter 7 Treatment Of
    96 TREATMENT OF SPECIFIC 7 HEMORRHAGES Johnny Mahlangu1 | Gerard Dolan2 | Alison Dougall3 | Nicholas J. Goddard4 | Enrique D. Preza Hernández5 | Margaret V. Ragni6 | Bradley Rayner7 | Jerzy Windyga8 | Glenn F. Pierce9 | Alok Srivastava10 1 Department of Molecular Medicine and Haematology, University of the Witwatersrand, National Health Laboratory Service, Johannesburg, South Africa 2 Guy’s and St. Thomas’ Hospitals NHS Foundation Trust, London, UK 3 Special Care Dentistry Division of Child and Public Dental Health, School of Dental Science, Trinity College Dublin, Dublin Dental University Hospital, Dublin, Ireland 4 Department of Trauma and Orthopaedics, Royal Free Hospital, London, UK 5 Mexico City, Mexico 6 Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA 7 Cape Town, South Africa 8 Department of Hemostasis Disorders and Internal Medicine, Laboratory of Hemostasis and Metabolic Diseases, Institute of Hematology and Transfusion Medicine, Warsaw, Poland 9 World Federation of Hemophilia, Montreal, QC, Canada 10 Department of Haematology, Christian Medical College, Vellore, India All statements identified as recommendations are • In general, the main treatment for bleeding episodes in consensus based, as denoted by CB. patients with severe hemophilia is prompt clotting factor replacement therapy and rehabilitation. However, different types of bleeds and bleeding at particular anatomical sites 7.1 Introduction may require more specific management with additional
    [Show full text]
  • Factor XIII Deficiency
    Factor XIII deficiency Information for families Great Ormond Street Hospital for Children NHS Foundation Trust 2 Factor XIII deficiency is a type of clotting disorder. A specific protein is missing from the blood so that injured blood vessels cannot heal in the usual way. This information sheet from Great Ormond Street Hospital (GOSH) explains the causes, symptoms and treatment of Factor XIII deficiency and where to get help. What is a clotting disorder? A clotting (or coagulation) disorder is a on in order. When all of the factors are turned medical condition where a specific protein on, the blood forms a clot which stops the is missing from the blood. injury site bleeding any further. Blood is made up of different types of There are a number of coagulation factors cells (red blood cells, white blood cells and circulating in the blood, lying in wait to be platelets) all suspended in a straw-coloured turned on when an injury occurs. If any one liquid called plasma. Platelets are the cells of the factors is missing from the body, the responsible for making blood clot. When complicated chemical reaction described a blood vessel is injured, platelets clump above will not happen as it should. This can together to block the injury site. They also lead to blood loss, which can be severe and start off a complicated chemical reaction to life-threatening. Each coagulation factor form a mesh made of a substance called fibrin. is given a number from I to XIII – they are This complicated chemical reaction always always written as Roman numerals – and follows a strict pattern – with each clotting the effects of the missing factor will vary.
    [Show full text]
  • The Rare Coagulation Disorders
    Treatment OF HEMOPHILIA April 2006 · No. 39 THE RARE COAGULATION DISORDERS Paula HB Bolton-Maggs Department of Haematology Manchester Royal Infirmary Manchester, United Kingdom Published by the World Federation of Hemophilia (WFH) © World Federation of Hemophilia, 2006 The WFH encourages redistribution of its publications for educational purposes by not-for-profit hemophilia organizations. In order to obtain permission to reprint, redistribute, or translate this publication, please contact the Communications Department at the address below. This publication is accessible from the World Federation of Hemophilia’s web site at www.wfh.org. Additional copies are also available from the WFH at: World Federation of Hemophilia 1425 René Lévesque Boulevard West, Suite 1010 Montréal, Québec H3G 1T7 CANADA Tel. : (514) 875-7944 Fax : (514) 875-8916 E-mail: [email protected] Internet: www.wfh.org The Treatment of Hemophilia series is intended to provide general information on the treatment and management of hemophilia. The World Federation of Hemophilia does not engage in the practice of medicine and under no circumstances recommends particular treatment for specific individuals. Dose schedules and other treatment regimes are continually revised and new side effects recognized. WFH makes no representation, express or implied, that drug doses or other treatment recommendations in this publication are correct. For these reasons it is strongly recommended that individuals seek the advice of a medical adviser and/or to consult printed instructions provided by the pharmaceutical company before administering any of the drugs referred to in this monograph. Statements and opinions expressed here do not necessarily represent the opinions, policies, or recommendations of the World Federation of Hemophilia, its Executive Committee, or its staff.
    [Show full text]
  • Protein C Deficiency
    Protein C deficiency Description Protein C deficiency is a disorder that increases the risk of developing abnormal blood clots; the condition can be mild or severe. Individuals with mild protein C deficiency are at risk of a type of blood clot known as a deep vein thrombosis (DVT). These clots occur in the deep veins of the arms or legs, away from the surface of the skin. A DVT can travel through the bloodstream and lodge in the lungs, causing a life-threatening blockage of blood flow known as a pulmonary embolism (PE). While most people with mild protein C deficiency never develop abnormal blood clots, certain factors can add to the risk of their development. These factors include increased age, surgery, inactivity, or pregnancy. Having another inherited disorder of blood clotting in addition to protein C deficiency can also influence the risk of abnormal blood clotting. In severe cases of protein C deficiency, infants develop a life-threatening blood clotting disorder called purpura fulminans soon after birth. Purpura fulminans is characterized by the formation of blood clots in the small blood vessels throughout the body. These blood clots block normal blood flow and can lead to localized death of body tissue ( necrosis). Widespread blood clotting uses up all available blood clotting proteins. As a result, abnormal bleeding occurs in various parts of the body, which can cause large, purple patches on the skin. Individuals who survive the newborn period may experience recurrent episodes of purpura fulminans. Frequency Mild protein C deficiency affects approximately 1 in 500 individuals. Severe protein C deficiency is rare and occurs in an estimated 1 in 4 million newborns.
    [Show full text]
  • Familial Multiple Coagulation Factor Deficiencies
    Journal of Clinical Medicine Article Familial Multiple Coagulation Factor Deficiencies (FMCFDs) in a Large Cohort of Patients—A Single-Center Experience in Genetic Diagnosis Barbara Preisler 1,†, Behnaz Pezeshkpoor 1,† , Atanas Banchev 2 , Ronald Fischer 3, Barbara Zieger 4, Ute Scholz 5, Heiko Rühl 1, Bettina Kemkes-Matthes 6, Ursula Schmitt 7, Antje Redlich 8 , Sule Unal 9 , Hans-Jürgen Laws 10, Martin Olivieri 11 , Johannes Oldenburg 1 and Anna Pavlova 1,* 1 Institute of Experimental Hematology and Transfusion Medicine, University Clinic Bonn, 53127 Bonn, Germany; [email protected] (B.P.); [email protected] (B.P.); [email protected] (H.R.); [email protected] (J.O.) 2 Department of Paediatric Haematology and Oncology, University Hospital “Tzaritza Giovanna—ISUL”, 1527 Sofia, Bulgaria; [email protected] 3 Hemophilia Care Center, SRH Kurpfalzkrankenhaus Heidelberg, 69123 Heidelberg, Germany; ronald.fi[email protected] 4 Department of Pediatrics and Adolescent Medicine, University Medical Center–University of Freiburg, 79106 Freiburg, Germany; [email protected] 5 Center of Hemostasis, MVZ Labor Leipzig, 04289 Leipzig, Germany; [email protected] 6 Hemostasis Center, Justus Liebig University Giessen, 35392 Giessen, Germany; [email protected] 7 Center of Hemostasis Berlin, 10789 Berlin-Schöneberg, Germany; [email protected] 8 Pediatric Oncology Department, Otto von Guericke University Children’s Hospital Magdeburg, 39120 Magdeburg, Germany; [email protected] 9 Division of Pediatric Hematology Ankara, Hacettepe University, 06100 Ankara, Turkey; Citation: Preisler, B.; Pezeshkpoor, [email protected] B.; Banchev, A.; Fischer, R.; Zieger, B.; 10 Department of Pediatric Oncology, Hematology and Clinical Immunology, University of Duesseldorf, Scholz, U.; Rühl, H.; Kemkes-Matthes, 40225 Duesseldorf, Germany; [email protected] B.; Schmitt, U.; Redlich, A.; et al.
    [Show full text]
  • Immune-Pathophysiology and -Therapy of Childhood Purpura
    Egypt J Pediatr Allergy Immunol 2009;7(1):3-13. Review article Immune-pathophysiology and -therapy of childhood purpura Safinaz A Elhabashy Professor of Pediatrics, Ain Shams University, Cairo Childhood purpura - Overview vasculitic disorders present with palpable Purpura (from the Latin, purpura, meaning purpura2. Purpura may be secondary to "purple") is the appearance of red or purple thrombocytopenia, platelet dysfunction, discolorations on the skin that do not blanch on coagulation factor deficiency or vascular defect as applying pressure. They are caused by bleeding shown in table 1. underneath the skin. Purpura measure 0.3-1cm, A thorough history (Table 2) and a careful while petechiae measure less than 3mm and physical examination (Table 3) are critical first ecchymoses greater than 1cm1. The integrity of steps in the evaluation of children with purpura3. the vascular system depends on three interacting When the history and physical examination elements: platelets, plasma coagulation factors suggest the presence of a bleeding disorder, and blood vessels. All three elements are required laboratory screening studies may include a for proper hemostasis, but the pattern of bleeding complete blood count, peripheral blood smear, depends to some extent on the specific defect. In prothrombin time (PT) and activated partial general, platelet disorders manifest petechiae, thromboplastin time (aPTT). With few exceptions, mucosal bleeding (wet purpura) or, rarely, central these studies should identify most hemostatic nervous system bleeding;
    [Show full text]
  • Outcomes of Patients with Thrombocytopenia Evaluated at Hematology Subspecialty Clinics
    Henry Ford Health System Henry Ford Health System Scholarly Commons Hematology Oncology Articles Hematology-Oncology 2-11-2021 Outcomes of patients with thrombocytopenia evaluated at hematology subspecialty clinics Zaid H. Abdel Rahman Kevin C. Miller H Jabbour Yaser Alkhatib Vijayalakshmi Donthireddy Follow this and additional works at: https://scholarlycommons.henryford.com/ hematologyoncology_articles Hematol Oncol Stem Cell Ther xxx (xxxx) xxx Available at www.sciencedirect.com ScienceDirect journal homepage: www.elsevier.com/locate/hemonc Outcomes of patients with thrombocytopenia evaluated at hematology subspecialty clinics Zaid H. Abdel Rahman a,*, Kevin C. Miller b, Hiba Jabbour c, Yaser Alkhatib c, Vijaya Donthireddy c a Division of Hematology and Medical Oncology, Mayo Clinic, Jacksonville, FL, USA b Department of Medicine, Massachusetts General Hospital, Boston, MA, USA c Division of Hematology and Medical Oncology, Henry Ford Hospital, Detroit, MI, USA Received 6 October 2020; received in revised form 9 December 2020; accepted 15 January 2021 KEYWORDS Abstract Hematology; Background: Thrombocytopenia is a frequently encountered laboratory abnormality and a Malignancy; common reason for hematology referrals. Workup for thrombocytopenia is not standardized Platelets; and frequently does not follow an evidence-based algorithm. We conducted a systematic anal- Referrals; Thrombocytopenia ysis to evaluate the laboratory testing and outcomes of patients evaluated for thrombocytope- nia at hematology clinics in a tertiary referral center between 2013 and 2016. Patient and methods: We performed a comprehensive chart review for patients evaluated for thrombocytopenia during the study period. Patients were followed for 1 year from the initial hematology evaluation and assessed for the development of a hematologic malignancy, rheumatologic, or infectious diseases among other clinical outcomes.
    [Show full text]
  • The Approach to Thrombosis Prevention Across the Spectrum of Philadelphia-Negative Classic Myeloproliferative Neoplasms
    Review The Approach to Thrombosis Prevention across the Spectrum of Philadelphia-Negative Classic Myeloproliferative Neoplasms Steffen Koschmieder Department of Medicine (Hematology, Oncology, Hemostaseology, and Stem Cell Transplantation), Faculty of Medicine, RWTH Aachen University, Pauwelsstr. 30, D-52074 Aachen, Germany; [email protected]; Tel.: +49-241-8080981; Fax: +49-241-8082449 Abstract: Patients with myeloproliferative neoplasm (MPN) are potentially facing diminished life expectancy and decreased quality of life, due to thromboembolic and hemorrhagic complications, progression to myelofibrosis or acute leukemia with ensuing signs of hematopoietic insufficiency, and disturbing symptoms such as pruritus, night sweats, and bone pain. In patients with essential thrombocythemia (ET) or polycythemia vera (PV), current guidelines recommend both primary and secondary measures to prevent thrombosis. These include acetylsalicylic acid (ASA) for patients with intermediate- or high-risk ET and all patients with PV, unless they have contraindications for ASA use, and phlebotomy for all PV patients. A target hematocrit level below 45% is demonstrated to be associated with decreased cardiovascular events in PV. In addition, cytoreductive therapy is shown to reduce the rate of thrombotic complications in high-risk ET and high-risk PV patients. In patients with prefibrotic primary myelofibrosis (pre-PMF), similar measures are recommended as in those with ET. Patients with overt PMF may be at increased risk of bleeding and thus require a more individualized approach to thrombosis prevention. This review summarizes the thrombotic Citation: Koschmieder, S. The risk factors and primary and secondary preventive measures against thrombosis in MPN. Approach to Thrombosis Prevention across the Spectrum of Keywords: myeloproliferative neoplasms (MPN); polycythemia vera (PV); essential thrombocythemia Philadelphia-Negative Classic (ET); primary myelofibrosis (PMF); thrombosis; prevention; antiplatelet agents; anticoagulation; cy- Myeloproliferative Neoplasms.
    [Show full text]
  • Severe Fever with Thrombocytopenia Syndrome: a Newly Discovered Emerging Infectious Disease
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector REVIEW Severe fever with thrombocytopenia syndrome: a newly discovered emerging infectious disease D. X. Li Key Laboratory for Medical Virology, National Institute for Viral Disease Control and Prevention, China CDC, Beijing, China Abstract Severe fever with thrombocytopenia syndrome (SFTS) is a newly discovered emerging infectious disease that has recently become epidemic in Asia. The causative agent of SFTS is a novel phlebovirus in the family Bunyaviridae, designated SFTS virus (SFTSV). SFTS clinically presents with high fever, thrombocytopenia, leukocytopenia, gastrointestinal disorders, and multi-organ dysfunction, with a high viral load and a high case- fatality rate. In human infection, SFTSV targets microphages, replicates in the spleen of infected mice, and causes thrombocytopenia and a cytokine storm. The tick disseminates virus to humans and animals, forming a special transmission model in nature. Person-to-person transmission though direct contact with patient blood has been frequently reported. Measurements of viral RNA and antibodies have been established for diagnosis, but vaccines and specific therapeutics are not available so far. Clinical Microbiology and Infection © 2015 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved. Keywords: Clinical, epidemiology, SFTS virus, SFTS, virology Article published online: 11 March 2015 Virology D.X. Li, Key Laboratory for Medical Virology, NCHFP, RRC, National Institute for Viral Disease Control and Prevention, China CDC, Bei- jing 102206, China E-mail: [email protected] The causative agent of SFTS is SFTSV, which is a tick-borne virus in the family Bunyaviridae, genus Phlebovirus.
    [Show full text]
  • ISTH Couverture 6.6.2012 10:21 Page 1 ISTH Couverture 6.6.2012 10:21 Page 2 ISTH Couverture 6.6.2012 10:21 Page 3 ISTH Couverture 6.6.2012 10:21 Page 4
    ISTH Couverture 6.6.2012 10:21 Page 1 ISTH Couverture 6.6.2012 10:21 Page 2 ISTH Couverture 6.6.2012 10:21 Page 3 ISTH Couverture 6.6.2012 10:21 Page 4 ISTH 2012 11.6.2012 14:46 Page 1 Table of Contents 3 Welcome Message from the Meeting President 3 Welcome Message from ISTH Council Chairman 4 Welcome Message from SSC Chairman 5 Committees 7 ISTH Future Meetings Calendar 8 Meeting Sponsors 9 Awards and Grants 2012 12 General Information 20 Programme at a Glance 21 Day by Day Scientific Schedule & Programme 22 Detailed Programme Tuesday, 26 June 2012 25 Detailed Programme Wednesday, 27 June 2012 33 Detailed Programme Thursday, 28 June 2012 44 Detailed Programme Friday, 29 June 2012 56 Detailed Programme Saturday, 30 June 2012 68 Hot Topics Schedule 71 ePoster Sessions 97 Sponsor & Exhibitor Profiles 110 Exhibition Floor Plan 111 Congress Centre Floor Plan www.isth.org ISTH 2012 11.6.2012 14:46 Page 2 ISTH 2012 11.6.2012 14:46 Page 3 WelcomeCommittees Messages Message from the ISTH SSC 2012 Message from the ISTH Meeting President Chairman of Council Messages Dear Colleagues and Friends, Dear Colleagues and Friends, We warmly welcome you to the elcome It is my distinct privilege to welcome W Scientific and Standardization Com- you to Liverpool for our 2012 SSC mittee (SSC) meeting of the Inter- meeting. national Society on Thrombosis and Dr. Cheng-Hock Toh and his col- Haemostasis (ISTH) at Liverpool’s leagues have set up a great Pro- UNESCO World Heritage Centre waterfront! gramme aiming at making our off-congress year As setting standards is fundamental to all quality meeting especially attractive for our participants.
    [Show full text]