Transfusion Medicine & Blood Products
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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. -
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. -
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. -
Autosomal Recessive Inherited Bleeding Disorders in Pakistan
Naz et al. Orphanet Journal of Rare Diseases (2017) 12:66 DOI 10.1186/s13023-017-0620-6 RESEARCH Open Access Autosomal recessive inherited bleeding disorders in Pakistan: a cross-sectional study from selected regions Arshi Naz1* , Muhammad Younus Jamal1, Samina Amanat2, Ikram Din ujjan3, Akber Najmuddin4, Humayun Patel1, Fazle Raziq5, Nisar Ahmed6, Ayisha Imran7 and Tahir Sultan Shamsi1 Abstract Background: Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V & VIII, Von Willebrand Disease (vWD) type 3, Glanzmann’s thrombasthenia (GT) and Bernard–Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened for ARBDs. Prothrombin (PT), activated partial thromboplastin time (APTT), bleeding time (BT) and fibrinogen levels were measured. Cases with isolated prolonged APTT were tested for factors VIII and IX using factor assays This was followed by FXI:C level assessment in cases with normal FVIII and FIX levels. vWD was screened in patients with low FVIII levels. Factors II, V and X were tested in patients with simultaneous prolongation of PT and APTT. Peripheral blood film examination and platelet aggregation studies were performed to assess platelet disorders. Urea clot solubility testing was done to detect Factor XIII levels where platelet function tests were normal. Descriptive analysis was done using SPSS version 16. Results: Of the 429 suspected bleeding disorder patients, 148 (35%) were diagnosed with hemophilia A and 211 (49.1%) patients had ARBDs. 70 patients (16.3%) remained undiagnosed. Out of 211 patients with ARBD; 95 (33.8%) had vWD type 3. -
Stem Cells and Artificial Substitutes Could Ease the Dependence on Blood Donations
OUTLOOK BLOOD DOING WITHOUT DONORS Stem cells and artificial substitutes could ease the dependence on blood donations. ANDREW BAKER BY ELIE DOLGIN S12 | NATURE | VOL 549 | 28 SEPTEMBER©2017 Ma c2017millan Publishers Li mited, part of Spri nger Nature. All ri ghts reserved. ©2017 Mac millan Publishers Li mited, part of Spri nger Nature. All ri ghts reserved. BLOOD OUTLOOK ach year, at about 13,000 collection centres worldwide, phlebotomists stick needles in the veins of healthy vol- unteers and amass in excess of 110 million donations of blood. The volume collected is enough to fill 20 Olympic- sized swimming pools — but it’s nowhere near to meeting the medical demand for whole blood or its components. To fill the gap, an enterprising group of stem-cell biologists and bio- Eengineers hopes to produce a safe, reliable and bottomless supply of on-demand blood substitutes in the laboratory. According to Robert Lanza, a pioneer of stem cell therapies and head IMAGES IWM VIA GETTY CHETWYN/ SGT. of global regenerative medicine at Astellas Pharma in Marlborough, Massachusetts, current technologies are not yet ready to compete with the real stuff. “We’re not going to put blood banks out of business any time soon,” he says. But in the near future, artificial blood products could be approved for use when transfusions are not otherwise an option, such as during combat or in people with a religious objection to receiving blood transfusions. And therapies that rely on reprogrammed stem cells to produce components of blood might also help transfusion centres to relieve shortages or to avoid donor-derived contamination. -
Blood Product Replacement: Obstetric Hemorrhage
CMQCC OBSTETRIC HEMORRHAGE TOOLKIT Version 2.0 3/24/15 BLOOD PRODUCT REPLACEMENT: OBSTETRIC HEMORRHAGE Richard Lee, MD, Los Angeles County and University of Southern California Medical Center Laurence Shields, MD, Marian Regional Medical Center/Dignity Health Holli Mason, MD, Cedars-Sinai Medical Center Mark Rollins, MD, PhD, University of California, San Francisco Jed Gorlin, MD, Innovative Blood Resources/Memorial Blood Center, St. Paul, Minnesota Maurice Druzin, MD, Lucile Packard Children’s Hospital Stanford University Jennifer McNulty, MD, Long Beach Memorial Medical Center EXECUTIVE SUMMARY • Outcomes are improved with early and aggressive intervention. • Both emergency blood release and massive transfusion protocols should be in place. • In the setting of significant obstetric hemorrhage, resuscitation transfusion should be based on vital signs and blood loss and should not be delayed by waiting for laboratory results. • Calcium replacement will often be necessary with massive transfusion due to the citrate used for anticoagulation in blood products. • During massive transfusion resuscitation, the patient’s arterial blood gas, electrolytes, and core temperature should be monitored to guide clinical management and all transfused fluids should be warmed; direct warming of the patient should be initiated as needed to maintain euthermia and to avoid added coagulopathy. BACKGROUND AND LITERATURE REVIEW After the first several units of packed red blood cells (PRBCs) and in the face of continuing or worsening hemorrhage, aggressive transfusion therapy becomes critical. This report covers the experience with massive transfusion protocols. Lessons from military trauma units as well as civilian experience with motor vehicle accidents and massive obstetric hemorrhage have identified new principles such as earlier use of plasma (FFP/thawed plasma/plasma frozen within 24 hours/liquid plasma) and resuscitation transfusion while laboratory results are pending. -
Platelet-Rich Plasmapheresis: a Meta-Analysis of Clinical Outcomes and Costs
THE jOURNAL OF EXTRA-CORPOREAL TECHNOLOGY Original Article Platelet-Rich Plasmapheresis: A Meta-Analysis of Clinical Outcomes and Costs Chris Brown Mahoney , PhD Industrial Relations Center, Carlson School of Management, University of Minnesota, Minneapolis, MN Keywords: platelet-rich plasmapheresis, sequestration, cardiopulmonary bypass, outcomes, economics, meta-analysis Presented at the American Society of Extra-Corporeal Technology 35th International Conference, April 3-6, 1997, Phoenix, Arizona ABSTRACT Platelet-rich plasmapheresis (PRP) just prior to cardiopulmonary bypass (CPB) surgery is used to improve post CPB hemostasis and to minimize the risks associated with exposure to allogeneic blood and its components. Meta-analysis examines evidence ofPRP's impact on clinical outcomes by integrating the results across published research studies. Data on clinical outcomes was collected from 20 pub lished studies. These outcomes, DRG payment rates, and current national average costs were used to examine the impact of PRP on costs. This study provides evidence that the use of PRP results in improved clinical outcomes when compared to the identical control groups not receiving PRP. These improved clinical out comes result in subsequent lower costs per patient in the PRP groups. All clinical outcomes analyzed were improved: blood product usage, length of stay, intensive care stay, time to extu bation, incidence of cardiovascular accident, and incidence of reoperation. The most striking differences occur in use of all blood products, particularly packed red blood cells. This study provides an example of how initial expenditure on technology used during CPB results in overall cost savings. Estimated cost savings range from $2,505.00 to $4,209.00. -
Blood Product Modifications: Leukofiltration, Irradiation and Washing
Blood Product Modifications: Leukofiltration, Irradiation and Washing 1. Leukocyte Reduction Definitions and Standards: o Process also known as leukoreduction, or leukofiltration o Applicable AABB Standards, 25th ed. Leukocyte-reduced RBCs At least 85% of original RBCs < 5 x 106 WBCs in 95% of units tested . Leukocyte-reduced Platelet Concentrates: At least 5.5 x 1010 platelets in 75% of units tested < 8.3 x 105 WBCs in 95% of units tested pH≥6.2 in at least 90% of units tested . Leukocyte-reduced Apheresis Platelets: At least 3.0 x 1011 platelets in 90% of units tested < 5.0 x 106 WBCs 95% of units tested pH≥6.2 in at least 90% of units tested Methods o Filter: “Fourth-generation” filters remove 99.99% WBCs o Apheresis methods: most apheresis machines have built-in leukoreduction mechanisms o Less efficient methods of reducing WBC content . Washing, deglycerolizing after thawing a frozen unit, centrifugation . These methods do not meet requirement of < 5.0 x 106 WBCs per unit of RBCs/apheresis platelets. Types of leukofiltration/leukoreduction o “Pre-storage” . Done within 24 hours of collection . May use inline filters at time of collection (apheresis) or post collection o “Pre-transfusion” leukoreduction/bedside leukoreduction . Done prior to transfusion . “Bedside” leukoreduction uses gravity-based filters at time of transfusion. Least desirable given variability in practice and absence of proficiency . Alternatively performed by transfusion service prior to issuing Benefits of leukoreduction o Prevention of alloimmunization to donor HLA antigens . Anti-HLA can mediate graft rejection and immune mediated destruction of platelets o Leukoreduced products are indicated for transplant recipients or patients who are likely platelet transfusion dependent o Prevention of febrile non-hemolytic transfusion reactions (FNHTR) . -
Factor XIII Deficiency
FACTSHEET Factor XIII deficiency This factsheet is about a bleeding disorder parents. It affects men and women equally. that is related to problems with a blood clotting factor called factor XIII (pronounced If you carry one copy of the gene fault for factor 13). It is written to go with our Rare factor XIII deficiency, you are known as a bleeding disorders booklet, where you will carrier. You can only pass the condition on to find much more information on living with your children if your partner also carries the one of these conditions. gene fault. You will not have the condition yourself, but any children that inherit the What is factor XIII deficiency? gene fault from you will also be carriers of the condition. Factor XIII deficiency is a bleeding disorder caused by the body producing less of a It is also possible to develop factor XIII clotting factor than it should. This causes deficiency later in life. This is called acquired problems because the clotting reaction factor XIII deficiency. It can be caused by that would normally control any bleeding liver disease, some types of leukaemia, is blocked too early. So your body doesn’t inflammatory bowel disease and an auto- make the blood clots it needs to stop immune disease called systemic lupus bleeding. erythematosus. Factor XIII deficiency is one of the rarest Symptoms of factor XIII deficiency types of clotting disorder. Doctors estimate that it affects about one in every two million Often, the first clinical sign of inherited people. Factor XIII plays an important role in factor XIII deficiency is a few days after wound healing, pregnancy and formation of birth or when the umbilical cord separates. -
Red Blood Cells, Leukocyte Reduced Covenant and AHS Sites
This document applies to all Red Blood Cells, Leukocyte Reduced Covenant and AHS sites. Class: Human blood component, derived from OTHER NAMES: Packed red blood cells, Packed whole blood cells INTRAVENOUS OTHER ROUTES DIRECT IV Intermittent Continuous Infusion Infusion SC IM OTHER Acceptable No Yes No No No No Routes* * Professionals performing these restricted activities have received authorization from their regulatory college and have the knowledge and skill to perform the skill competently. DESCRIPTION OF PRODUCT: Red Blood Cell (RBC) concentrates are prepared from approximately 480mL whole blood usually collected in 70mL of anticoagulant. The unit is plasma reduced by centrifugation, platelet reduced by either centrifugation or filtration and leukocyte reduced by filtration. RBCs are typically resuspended in approximately 110mL of nutrient. Collected from volunteer donors by the Canadian Blood Services (CBS). Donor is screened and blood is tested for: i. Hepatitis B Surface Antigen (HBsAg) ii. Syphilis iii. Antibodies to Hepatitis B core antigen (HBcore), Hepatitis C Virus (HCV), Human T-cell Lymphotropic Virus (HTLV-1 and 2), Human Immune Deficiency Virus (HIV-1 and 2) iv. Nucleic Acid Testing (NAT) for presence of HCV RNA, HIV-1 RNA and West Nile Virus (WNV) RNA . CBS also tests blood for ABO/Rh and clinically significant antibodies. Some donors are tested for CMV status. RBCs do NOT contain any coagulation factors or platelets. AVAILABILITY: . Contact your local transfusion service/laboratory for ABO and Rh types stocked at your site. ABO compatible (not always identical) may be required if the transfusion service cannot provide sufficient quantities of the patient’s blood group (See ABO compatibility chart at: http://www.albertahealthservices.ca/assets/wf/lab/wf-lab-clin-tm-abo-compatability.pdf). -
CCEMS Whole Blood Protocol
PROCEDURES 31 Revised 8/2016 Assessment Approved: Levon Vartanian, MD Hemoglobin Assessment Indications Equipment Needed Trauma or medical patient with suspected blood CLIAwaived photometer, alcohol swab, gauze loss pad, lancet, Capillary Transfer Tube, Test cartridges Procedure 1. Make sure the meter is ready for use. Turn meter on, and when the on-screen display indicates, insert the testing strip all the way in. 2. Cleanse site for capillary puncture using alcohol pad and allow to dry. 3. Lance side of fingertip. Blot away first drop of blood. 4. Press gently once again to get a 2nd drop of blood. 5. Place the tip of the capillary collection tube against the drop of blood. DO NOT squeeze capillary collection tube. 6. Allow capillary collection tube to draw up blood to the level of the black line. 7. Apply blood sample to the specimen area on the test strip. It should fill the entire test area with blood. 8. The meter will then read hemoglobin and hematocrit values. 9. Document findings in PCR. Dispose of microcuvette and lancet into sharps container. Pearls • The meter requires a code chip packaged with each box of testing strips. This chip must be inserted on the left hand side of the meter in slot provided. • Only capillary blood may be tested. Do not use arterial or venous blood. • Samples should be immediately tested. • Hematocrit values will not display if hemoglobin values are outside of the range 12.3–17.5 g/Dl. • Meter will read LO if test result is < 4.5 g/Dl OR if insufficient blood was applied. -
Red Blood Cell Transfusions –
Peer Reviewed VETcpd - Internal Medicine Jenny Walton BVM&S MRCVS Jenny qualified from R(D)SVS in Red blood cell transfusions – 1998 - she worked in mixed practice when, what and how to do it! for 4 years before moving into the Red cell transfusions are now a relatively common intervention in veterinary practice field of small in the UK and help in the treatment of many patients. This is largely due to the animal emergency availability of blood products, such as Packed Red Blood Cells (PRBC) , from blood and critical care banks supplying directly to practices. After donation, red cells are separated from with Vets Now for 12 years. Through plasma into a concentrated packed cell form, a nutrient extender is then added to Vets Now, she ran the practical trial them. This allows the red cells to be stored for up to 42 days before being transfused researching canine blood banking in 2005-2006 – launching Pet Blood Bank into patients. DEA 1 blood typing prior to transfusion is essential and cross matching UK (PBB) alongside Wendy Barnett should be performed for second transfusions. Blood products are administered in 2007. She acts as the Veterinary through a filtered giving set and patients monitored closely for transfusion reactions. Supervisor for PBB, her role includes Transfusion reactions are thankfully rare but potentially life threatening. advising practitioners daily on the appropriate use of PBB blood products, Key words: Canine, Packed Red Blood Cells (PRBC), transfusion, blood typing, overseeing the practical and VMD cross matching, transfusion reactions legislative veterinary aspects of blood collection at PBB and leading research on future development opportunities.