Hematology: Basic Surgery Notes PDF

BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. ⑨ Disclaimer: Pakibasa nang may pag-intindi ang first 4 pages...

BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. ⑨ Disclaimer: Pakibasa nang may pag-intindi ang first 4 pages VASCULAR CONSTRICTION para ma-gets ang remaining pages. Thank you! Initial response to vessel injury More pronounced in vessels w/ medial smooth muscle wall BIOLOGY OF HEMOSTASIS Hemostasis o Process that retains blood within the vascular system during periods of injury, localizes the reactions involved to the site of injury, and repairs and re-establishes blood flow; through the injure vessel (Ty Lemar) o Complex process whose function is to limit blood loss from an injured vessel o Combined interaction and interplay between platelets, endothelium, and coagulation factors Primary action ng hemostasis ay limit blood loss kapag, kapag nagkaroon ng injury dapat merong hemostasis kasi kapag hindi balance its either magkakaroon ng severe blood loss or clot formation. So kailangan balance ang clot formation at blood loss to achieve hemostasis. 1. Thromboxane A2 (TXA2) from At 2. Endothelin and 5 HT 3. Serotonin 4. Bradykinin and fibrinopeptides potent vasoconstrictors Ang vasoconstriction ay initiated by TXA2, Serotonin, endothelin basta yang apat na yan. ⑬ PLATELET FUNCTION Anucleate fragments of megakaryocytes 150,000 – 400.000/μL (normal value) 30% sequestered in the spleen (70% is in the circulation) Average life span is 7-10 days fragile VASOCONSTRICTION → PRIMARY HEMOSTASIS → GPIDG O GPIbB SECONDARY HEMOSTASIS → FIBRINOLYSIS GPIX & of Biology of hemostasis. The four physiologic processes that interrelate to limit blood loss from an injured vessel are illustrated and include vascular constriction, platelet plug formation, fibrin clot formation, and fibrinolysis. = Memorize lang ‘tong mga values kasi kapag affected ang Step by step ang hemostasis kita naman sa picture diba? Siymepre una is vasoconstriction para hindi na magleak ung blood, and then value ng platelet whether quantitative or qualitative may mga papasok na si platelet para magaggregate forming now yung diseases na pwedeng mag-arise. tinatawag na initial platelet plug yan yung tinatawag na primary hemostasis since initial lang yon madali siyang matatanggal so ② PLATELET FUNCTION mangyayari magkakaroon ng coagulation phase (eto na ung mga Play an integral role in hemostasis by forming a hemostatic cascade or secondary hemostasis) and finally ung sa part ng plug and thrombin formation fibrinolysis, kasi hindi pwedeng may andon lang yung clot na naform Platelets do not normally adhere to each other or to the so kapag magaling na ang sugat kailangan matanggal na yung clot vessel wall na nabuo. KUMUNOY’S IMPROPERTY 1  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. Ang normal shape ng platelet ay discoid tas kapag activated na magiging spherical ewan ko dito bat naging star? Explain natin to nang bongga sige. After magkaroon ng injury HAHAHAHA! Siyempre once activated ilalabas niya na yung to the tissue ang mga platelets pupunta yan sa site ng injury granules niya which will be further discussed, EME! (saan ba kasi yon pota, siyempre sa subendothelium which contains collagen and fibronectin) at sa subendothelium mag- PRIMARY HEMOSTASIS aadhere si platelet forming now initial hemostatic plug under Injury to the intimal layer of the vascular wall exposes sub- the primary hemostasis. Merong 4 steps ang primary endothelial collagen hemostasis: von Willebrand factor (vWf) binds GP I/IX/V 1. Platelet Adhesion Platelets initiate a release reaction recruiting other - Adhesion ng platelet sa subendothelium yan na ‘yon circulating platelets dense < granules beh hahaha, pero with the help of von willebrand factor (vWF) at ang kanyang receptor ay glycophorin 1B receptor (Gp1B). Tingin sa picture, para makapag adhere si platelet kailangan muna ng Gp1B at vWF kapag wala silang dalawa walang adhesion walang platelet plug formation magkakaroon ng blood loss. - No vWF → Von willebrands disease - No Gp1B → Bernard Soulier syndrome 2. Platelet Activation - Morphologic and functional changes in the platelets, from discoid to spherical in shape. - Yung platelets merong cyclooxygenase yan kapag nagging activated, si cyclo imemetablize niya si secretion - Platelet aggregation is REVERSIBLE and not associated w/ arachidonic acid para magiing prostaglandin the eventually para maging TXA2 which is a Heparin does not interfere with these reaction vasoconstrictor and platelet stimulator so parg ADP and serotonin are principal mediators nangyari to magkakaroon ng secretion at Second wave of platelet aggregation reaction occurs aggregation o ADP, Ca, fibrinogen, TXA2, ⍺-granule proteins stabilize clot - Aspirin inactivates the enzyme cyclooxygenase. o Fibrinogen is required cofactor acting as a bridge for GP IIb/IIIa receptor 3. Platelet secretion - Once nagkaroon na ng shape change, masesecrete - o Irreversible na yung granules ni platelet which are alpha at dense Thrombospondin strengthens platelet interaction and fibrinogen binding granules potent heparin for clot not to dissolve PF4 and ⍺-thromboglobulin are also released Alteration in the platelet membrane allows Ca and clotting 4. Platelet aggregation factors to bind (PF3) converting Prothrombinn to thrombin - Kapag mering tuloy-tuloy na secretion ng granules ng by activated Factor Xa in the presence of Factor V and Ca platelet nagccause ito na magkaroon ng platelet KUMUNOY’S IMPROPERTY 2  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. aggregation. Tingin sa pictue, diba merong fibrinogen, Yang picture na nasa taas, oo ganyan talaga kagulo yan pero siya yung nagiging bridge para mag adhere sa isat isa siyempre ano bang ginagawa ni kumunoy mas lalo nating ung mga platelets, at ang kanyang receptor ay is paguluhin potaena. Konting recap, hindi stable si primary Gp2B/3A. hemostasis kaya merong secondary, tong seconday is all - No Gp2B/3A → Glanzmann’s thrombasthenia about coagulation factor at and end product siyempre coagulation (clot formation). Merong tatlong pathway para After ng platelet aggregation, meron na tayong initial platelet makapagproduce ng coagulation: plug pero gaya ng sabi ko kanina unstable pa yang clot na yan so madaling masira, imagine kapa gung clot na tatakip sa 1. Intrinsic pathway by itself can initiate coagulation sugat is madaling matanggal magkakaroon uli ng blood loss - Kapag nagkaroon ng exposure ng surface ng so ang next na gagawin is secondary hemostasis para sa more endothelium, yun yong mag-iinitiate ng pathway. stable clot. Activating different clotting factors such factor 8,9, Q COAGULATION 11,12 2. Extrinsic pathway - Initiated by vascular injury, activating clotting factor 7 3. Common Pathway - Ito yung meeting place ni intrinsic at extrinsic pathway, magstart sa factor 10 - Associated clotting factors are 1,2,5,10, 13 - Ito yung end ng coagulation and makabuo ng fibrin in the process of fibrin polymerization (stable plug) Coagulation factors are also known as enzyme precursor or zymogens. Found in the plasma along with non- end-to-end enzymatic cofactors and calcium Blood factors are mostly produced in the liver and 00 site to site circulate in inactive precursor form Factor VII is inactive, factor VIIa is active (Kapag meron ng ⑪ FIBRINOLYSIS “A” and isang factor ibig sabihin nasa active form na ito) Feedback inhibition on the coagulation cascade Initiation, amplification, and propagation Thrombomodulin is presented by the endothelium acts a o TF exposure following subendothelial injury binds to VIIa “thrombin sink” binds to thrombin cleave of fibrinogen ( catalyzing activation of: Protein C & S inhibits factor V and VIII ▪ X to Xa Tissue plasminogen activator (tPA) is released from Initiation ▪ IX to IXa endothelium following injury o Which in turn activates V to Va (prothrombinase complex) o Platelets adhere to extracellular matrix components at I the site of injury platelet plug o Factor VIIIa/IXa, Va/Xa are assembled on the surfaces of activated platelets (thrombin burst) fibrinogen-fibrin VIIIa/IXa is 50x more effective at catalyzing Factor X than TF-VIIa Thrombin cleaves fibrinogen by 2 cleavage steps this facilitated by TAFI stabilizing the clot KUMUNOY’S IMPROPERTY 3  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. Diba ang dulo ng common pathway is fibrin polymerization, COAGULATION FACTOR DEFICIENCIES dito sa fibrinolysis idedegrade yung fibrin kasi para walang Deficiency Other name(s) thrombus formation, ang main activator is Tissue plasminogen Factor VIII Deficiency Hemophilia A activator (tPA) which is released sa endothelium after din ng Factor IX Deficiency Hemophilia B, akeChristmas injury. Tignan niyo yung picture sa taas, tPA acts on disease - plasminogen to become its active form plasmin na magiging Factor XI Deficiency Hemophilia C dahilan ng FIBRIN DEGRADATION PRODUCT Spontaneous bleeding is rare a APC consumes PAI-1 o Activated protein C = APC prevention ofactivation Prevalent Ashkenazi among Jewish o Type 1 plasminogen activator inhibitor = PAI population o Si APC kinakain niya si PAI so walang inhibitor si Clinical severity of hemophilia A and hemophilia B plasminogen so tuloy and fibrinolysis depends on the measurable level of factor VIII and IX in AT-III (Anti-thrombin III) neutralizes all of the serene the patient’s plasma proteases o Plasma factors indicates fibrinolysis FACTOR VII DEFICIENCY Clot lysis yields FDPs (D-dimers and E-nodules) fibrin degradation product - Rare autosomal recessive disorder Plasmin is inhibited by ⍺2 – antiplasmin x-linked to fibrin by Spontaneous bleeding is uncommon Factor XIII, also inhibited by tPA and urokinase Easy bruising and mucosal bleeding Larger clot fragments maybe incorporated into the clot Postoperative bleeding 30% of surgical procedures Treatment is w/ FFP or VIIa 1 concentrate FACTOR XIII DEFICIENCY Rare autosomal recessive disorder Associated w/ severe bleeding diathesis Male to Female ratio is 1:1 Associated w/ inflammatory bowel disease, hepatic failure. Myeloid leukemia Bleeding is delayed Risk of intracranial bleeding and spontaneous abortion VON WILLEBRAND’S DISEASE Most common congenital bleeding disorder Easy mucosal bleeding and bruising Quantitative or qualitative defect vWF mababa enough pero padget vWF is responsible for carrying factor VIII and platelet adhesion o Type I – quantitative defect o Type II – qualitative defect o Type III – total deficiency KUMUNOY’S IMPROPERTY 4  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. PLATELET FUNCTIONAL DEFECTS Disseminated Intravascular Coagulation (DIC) Related to platelet thrombi o Thrombocytopenic purpura o Hemolytic uremic syndrome IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP) Acute, short lived, and typically follows a viral illness in children destruction of platelets > thrombocytopenia - Gradual in onset, chronic, and no identifiable cause in adult Impaired platelet production and T-cell mediated immune response First line of treatment o Corticosteroid to stop inflamm o Intravenous immunoglobulin or anti-D immunoglobulin Second line of treatment o Splenectomy o Rituximab (anti-CD 20 monoclonal antibody) ↑ platelets SURFACE PROTEIN ABNORMALITY o Thrombopoietin (TPO) receptor agonist 7)) BM> - GLANZMANN THROMBASTHENIA Third line treatment o Rare autosomal recessive disorder o Thrombopoietin receptor agonist synthetic stimulants -> attaches to fibrin o GP IIb/IIIa complex is lacking or dysfunctional o Combination chemotherapy o Faulty platelet aggregation and subsequent bleeding o Platelet transfusions HEPARIN INDUCED THROMBOCYTOPENIA (HIT) BERNARD-SOULIER SYNDROME Drug induced immune thrombocytopenia o Defect GP Ib/IX/V receptor for vWF Platelet count falls 5-7 days after heparin is started or 1-2 o Failure of platelet adhesions after re-exposure o Platelet transfusions Platelet count falls below 100,000 or drops by 50% from baseline STORAGE POOL DISEASE Thrombocytopenia and intravascular thrombosis develop Most common Absence of thrombocytopenia does not preclude the Loss of dense granules G diagnosis o Storage sites for ADP, ATP, Ca, Inorganic phosphates Negative ELISA rules out HIT however, a positive result does o Most prevalent not confirm HIT o Hermansky-Pudlak syndrome Treatment is to stop heparin and begin alternative Loss of alpha granules anticoagulant e.g lepirudin, danaparoid, argatroban FAILURE OF PRODUCTION THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) Leukemia thrombocytopenia Result of platelet activation and formation of thrombi Myeloproliferative disorders hypercoag Large vWF molecules interact w/ platelets B12 or Folate deficiencies Thrombocytopenia, microangiopathic hemolytic anemia, Chemotherapy or radiation therapy 7 megakarnocytes fever, renal and neurologic symptoms Alcohol intoxication Schistocytes aids in the diagnosis Viral infection thrombocytopenia caused by dengue FFP and rituximab is indicated DECREASED SURVIVAL ADAM-S13 /Metalloproteinase) inhibition Immune mediated shorter life span of platelets HEMOLYTIC UREMIC SYNDROME (HUS) o Idiopathic thrombocytopenia (ITP) Secondary to Escherichia coli O157:H7 or Shiga toxin o Heparin induced thrombocytopenia producing bacteria o Autoimmune disorders/B-cell malignancies Renal failure can be seen o Secondary thrombocytopenia Neurologic symptoms are less frequent KUMUNOY’S IMPROPERTY 5  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. Metalloproteinase is normal ADAM-S1 is normal o Low fibrinogen level HIV, SLE, and certain drugs (ticlopidine, mitomycin C, o Elevated fibrin markers (FDPs. D-dimer, soluble fibrin gemcitabine) can develop features of TTP and HUS monomers) Discontinuation and plasmapheresis TREATMENT o Relive the patient’s primary causative mechanism SEQUESTRATION o Maintain adequate perfusion due to enlarged spleen Portal hypertension o Hemostatic factors should be replaced (FFP, Sarcoid cryoprecipitate, fibrinogen concentrate, platelet Lymphoma concentrates) Gaucher’s disease o Heparin Total body platelet mass is normal PRIMARY FIBRINOLYSIS Bleeding is less than anticipated because sequestered Acquired hypofibrinogenic state urokinase breaks down fibrin platelet can be mobilized to the circulation Prostate resection and extracorporeal bypass Platelet does not increase count as much Severity of bleeding is dependent on the concentration of breakdown products QUALITATIVE DISORDERS Antifibrinolytic agents may be used Impaired platelet function often accompanies thrombocytopenia but may also occur in the presence of MYELOPROLIFERATIVE DISEASE a normal platelet count POLYCYTHEMIA VERA Drugs that interfere with platelet function: Increased blood viscosity o Aspirin, clopidogrel, prasugrel, dipyridamole, and GP Increased platelet count IIb/IIIa inhibitors Increased tendency towards stasis thrombus formation Aspirin, clopidogrel, and prasugrel all IRREVERSIBLY inhibit platelet function. Thrombocytosis can be reduced: Quity - Massive transfusion tranfused of platelets poor blood > o Low dose aspirin alty Therapeutic platelet inhibitors interfere ↓ platelet fo o Phlebotomy 2 Disease states o Hydroxyurea o Myeloproliferative disorders o Monoclonal gammopathies COAGULOPATHY OF LIVER DISEASE o Liver disease Liver disease conditions of fibrin o Disturbance in coagulation mechanisms ACQUIRED HYPOFIBRINOGENEMIA o Increased bleeding risk and thrombotic risk DISSEMINATED INTRAVASCULAR COAGULATION (DIC) o Thrombocytopenia and impaired humoral coagulation Acquired syndrome characterized by activation of coagulation pathways HYPERCOAGULABLE STATE - HYPOCOAGULABLE STATE Excessive thrombin generation and diffuse formation of ▪ ↓Decreased ▪ V Decreased production of microthrombi production of protein - coagulation factors Consumption of platelets and depletion of coagulation - C and S ▪ ↓ Decreased absorption of factors resulting to diffuse bleeding a spontaneous ▪ ↓ Decreased - vitamin K Presence of an underlying condition is required for the antithrombin and ▪ ↓ Decreased production of - diagnosis plasminogen thrombopoietin - Embolization (brain matter, amniotic fluid, bone marrow) ▪ - Elevated vWF and ▪ Immune mediated Malignancy; Organ injury; Liver failure; Snake bites; factor VIII thrombocytopenia Aneurysms; Illicit drug; Transfusion reaction; Transplant ▪ Hypersplenism rejection; Sepsis Thrombocytopenia is most common cause YDIAGNOSIS o Hypersplenism o Inciting etiology o Reduced production of thrombopoietin (TPO) o Thrombocytopenia o Immune mediated destruction (biliary cirrhosis, hepatitis o Prolongation of prothrombin time C) KUMUNOY’S IMPROPERTY 6  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. COAGULOPATHY OF TRAUMA Phenylbutazone, clofibrate, TRAUMA INDUCED COAGULOPATHY (TIC) O ↑Warfarin effect anabolic steroids, L-thyroxine, Shock and tissue injury are the initiating factors ↓Warfarin requirements glucagons, amiodarone, o Activated protein C (APC) mediated clotting factor quinidine, cephalosporins deactivation Bleeding complications o Endothelial injury and “auto-heparinization” due to o Hematuria shedding of heparin sulfate and chondroitin sulfate o Soft tissue bleeding o Platelet dysfunction and fibrinolysis o Skin necrosis o Abdominal bleeding Hemorrhagic shock was previously though as an essential Vitamin K and prothrombin complex concentrate component but isolated TBI and pulmonary contusions Andexanet alpha, ciraparantag does not have a hemorrhagic component Protamine sulfate aPTT of less than 1.3 times control in heparinized patient and INR less than 1.5 times in patients on warfarin Eye and CNS surgery should not be performed ① surgery Local hemostasis Mechanical procedures o ~Direct digital pressure o vApplication of tourniquet o ~Ligature o -Gauze or laparotomy pads for larger areas o -Bone wax Thermal agents electrocotoly ? ACQUIRED COAGULATION INHIBITORS TOPICAL HEMOSTATIC AGENTS Anti-phospholipid syndromes (APLS) / Ideal hemostatic agents o Most common acquired coagulation inhibitors o Significant hemostatic action o Include lupus anticoagulant and anticardiolipin o Minimal tissue reactivity antibodies promote thrombosis o Non-antigenicity o Associated w/ venous or arterial thrombosis o Biodegradable o Antiphospholipid antibodies are common in patients w/ o Low cost systemic lupus but can also be seen in RA and Sjögren’s ↑ Absorbable agents syndrome o Gelatin foams (gelfoam) o Hallmark is prolonged aPTT in vitro, increased risk of o Oxidized cellulose (surgical) thrombosis in vivo o Microfibrillar collagens (avitene) ~ Biologic agents ANTICOAGULATION AND BLEEDING o Thrombin Spontaneous bleeding can be a consequence of o Fibrin sealants (floseal) anticoagulation therapy o Platelet sealants (vitagel) o Heparin, low molecular weight heparin o Warfarin REPLACEMENT THERAPY o Factor Xa inhibitors (rivaroxaban, apixaban, edoxaban) Human blood replacement therapy was accepted in the o Thrombin inhibitors (dabigatran) late 19th century Warfarin can be used for long term anticoagulation Landsteiner identified the major blood group A, B, O in 1900 Levine and Stetson followed with the concept of Rh Medications that can alter warfarin dosing grouping in 1939 Barbiturates, oral contraceptives, Whole blood was considered the standard until in the 1970s O↓Warfarin effect - estrogen-containing compounds, component therapy gain prominence ↑Warfarin requirements corticosteroids, Typing and crossmatching adrenocorticotropic hormone KUMUNOY’S IMPROPERTY 7  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. o Serologic compatibility between ABO and Rh group is Source factor V routine Can be thawed and stored for 5 days at 2-4 ºC o Major crossmatching is performed Freeze-dried (lyophilized) plasma (FDP) o Rh negative should only be transfused w/ Rh negative blood INDICATIONS FOR REPLACEMENT In emergency situation, type O negative RBC and AB Improvement in O2 carrying capacity plasma may be transfused to all recipients Treatment of anemia In patients with clinically significant cold agglutinins, blood o Hgb 34% ↓ V THR ▪ Amount of blood suctioned Up to 5u can be donated M/ ▪ Weighing of sponges 1st procurement can be made 40 days before the ▪ Amount of blood in the wound and drapes operation and the last one performed 3 days before o Blood loss of 20% can be replaced w/ crystalloid or colloid 10 fluids BANKED WHOLE BLOOD o >20% balance resuscitation Shelf life: 42 days Changes in the RBC DAMAGE CONTROL RESUSCITATION (DCR) o Reduction of intracellular ADP, 2,3-DPG Start resuscitation with crystalloid followed by PRBC only Stored RBCs become progressively acidotic after several liters of crystalloid before you transfuse o Lactate plasma or platelets o Potassium Three (3) basic components of DCR o Ammonia o~ Permissive hypotension o~ Minimize crystalloid and colloid based resuscitation FROZEN RED BLOOD CELLS o ~ Immediate release and administration of predefined Shelf life: 10 years at -80ºC blood products Used for patients that were previously sensitized o Plasma:Platelet:RBC ratio 1:1:1 – 0.3:0.1:1 w/ survival rates ATP and 2,3-DPG are maintained 71% to 41% LEUKOCYTE REDUCED / WASHED RBCs Leukocyte reduction prevents almost all febrile non- ADULT MASSIVE TRANSFUSION (CPG) hemolytic transfusion reactions, alloimmunization to HLA Component Therapy Administration During Massive Transfusion class 1 antigen, platelet transfusion refractoriness, Fresh frozen As soon as the need for massive ①cytomegalovirus plasma (FFP) transfusion is recognized For every 6 RBCs, give 6 FFP (1:1 ratio) PLATELET CONCENTRATES For every 6 RBCs and plasma, give one Thrombocytopenia and massive blood loss 6-pack platelets. 6 Random-donor Shelf life: 120 hours Platelets platelet packs = 1 apheresis platelet unit Platelet transfusions are capable of transfusing infectious Platelets are in every cooler Keep platelet counts >100,000 diseases After first 6 RBCs, check fibrinogen level. If ≤ 200mg/dL, give 20 units of FRESH FROZEN PLASMA Cryoprecipitate cryoprecipitate (2g fibrinogen) Shelf life: 2 years at -18ºC Repeat as needed, depending on Source of Vitamin K-dependent factors 9 factor V fibrinogen level, and request o Vitamin K dependent factors: Factors II, VII, IX, X, Protein appropriate amount of cryoprecipitate C, protein S KUMUNOY’S IMPROPERTY 8 TAKE IT EASY  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. COMPLICATIONS OF TRANSFUSION Hemolytic Transfusion reaction (Acute) Blood induced pro-inflammatory responses o Occur with the administration of ABO incompatible o Occurs in 10% of all transfusion, 1ºC body temperature Hemolytic Transfusion reaction (Delayed) o 1% of all transfusions o Occurs 2-10 days after transfusion o Can be greatly reduced w/ leukocyte washed RBCs o Extravascular hemolysis, mild anemia, and o Acetaminophen reduces severity of the reaction hyperbilirubinemia + o Occurs when an individual has a low tier at the time of M Bacterial contamination of infused blood administration o Gram negative blood capable of growth at 4ºC o Pain at the site of transfusion, facial flushing, back and o Platelets stored at 20ºC chest pain o FFP thawed in a contaminated water o IgG mediated o Results in sepsis and death in 25% o Positive (+) Coomb’s test o Fever/chills, tachycardia, hypotension, GI symptoms o Oxygen, adrenergic blocking agents, antibiotics Transfusion of disease o Malaria, Chaga’s disease, brucellosis, syphilis Allergic reactions o Hepatitis C, HIV, CMV o 1% of all transfusion o Creutzfeldt-Jakobs disease o Mild and consists of rash, urticaria, and flushing o Zika virus o Rare instances anaphylactic shock develops Which of the following occurs after rapid Transfusion- o Transfusion of antibodies from a hypersensitive donor or infusion of blood products which manifests associated transfusion of antigens as rise in venous pressure, dyspnea and circulatory overload o Commonly related with FFP and platelets cough? TRALI – Fever, rigors, o Antihistamines, epinephrine, steroids a. ABO Hemolytic transfusion reaction bilateral pulmonary b. Transfusion-related acute lung injury infiltrates on CXR TACO (Transfusion associated circulatory overload) c. Allergic reaction Allergic – rash, o Occurs with rapid infusion of blood, plasma expanders, d. Transfusion-associated circulatory urticaria, and and crystalloids overload flushing What factor is responsible for cross linking o More common in elderly patient w/ underlying heart alpha 2 antiplasmin to fibrin? disease XIII a. II b. XIII o Manifested as rise in venous pressure, dyspnea, and c. VIII d. V cough pulmonary edema What is the third phase of hemostasis? Clot formation o Rales can be heard at the lung bases I – vasoconstriction a. Platelet aggregation o Diuresis, slow rate of blood administration, minimize fluids II – platelets b. Fibrinolysis aggregate c. Vasoconstriction TRALI (Transfusion-related acute lung injury) III – clot formation d. Clot formation o Noncardiogenic pulmonary edema related transfusion IV – fibrinolysis Factor XIII deficiency associated dyspnea and hypoxemia Which rare autosomal recessive disease vWF – most common o Can occur with administration of any plasma containing that is usually associated with severe congenital bleeding blood product bleeding diathesis? disorder, easy mucosal bleeding and bruising o Fever, rigors, bilateral pulmonary infiltrates on CXR a. von Willebrand’s disease VII – rare autosomal o Commonly occurs 1-2 hrs after transfusion b. Factor IX deficiency recessive disorder, c. Factor VIII deficiency postoperative bleeding o Treatment is discontinuation of transfusion and d. Factor XIII deficiency 30%% of surgical pulmonary support procedures KUMUNOY’S IMPROPERTY 9  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. What substance is presented by the Thrombomodulin von Willebrand Which protein is responsible for platelet Thrombospondin factor endothelium and forms a complex with adhesion during injury to the sub strengthens platelet thrombin rendering it no longer available to P-selectin rolling interaction and fibrinogen endothelium? cleavage fibrinogen? binding GPVI activation and a. Glycoprotein VI a. Thrombospondin Activated Protein C aggregation of plt b. P Selectin b. Activated protein C Si APC kinakain niya si PAI GPIIB/IIIA regulation so walang inhibitor si c. Glycoprotein Iib/IIIa c. Thrombomodulin of platelet adhesion plasminogen so tuloy and d. von Willebrand factor d. Thrombin activatable fibrinolysis inhibitor and aggregation fibrinolysis Which of the following takes place during What is the most potent mechanism of the fourth phase of hemostasis? thrombin nhibition? a. All of the choices are correct All of the choices are a. TF – VIIa complex APC system b. Fibrin polymers are degraded by plasmin correct b. VIIIa – Ixa complex c. Restoration of blood flow c. Thrombin activatable fibrinolysis inhibitor d. Tissue Plasminogen activator d. APC system How is Thromboxane A2, potent Which of the following pathway is paired vasoconstrictor, produced locally at the correctly in relation to neuroendocrine Posterior pituitary: site of injury? Release of response to injury? Anti-diuretic a. Release of arachidonic acid by the arachidonic acid by a. Posterior pituitary: Anti-diuretic hormone hormone platelet membranes the platelet b. Anterior pituitary: Thyroid releasing “releasing hormone” b. Synthesized by the injured endothelium membranes hormone hypothalamus dapat c. Activation of Factor X c. Anterior pituitary: Corticotropin releasing d. Synthesized by the liver hormone FSH – anterior d. Posterior pituitary: Follicle stimulating pituitary Which of the following is associated with Thrombotic hormone microangiopathic hemolytic anemia and thrombocytopenic schistocytes in the peripheral blood More pronounced in purpura Which of the following statement/s is/are smear? SLE – antiphospholipid vessels with smooth TRUE for the first phase of hemostasis? a. Systemic lupus erythematosus antibodies muscles ITP – impaired platelet a. Platelet adhesion First phase is b. Hemolytic uremic syndrome b. All of the choices are correct production and T-cell vasoconstriction – mas c. Thrombotic thrombocytopenic purpura mediated immune c. von Willebrand factor is necessary for this pronounced sa vessels d. Idiopathic thrombocytopenic purpura response phase with medial smooth Which of the following statement is TRUE Begins with the d. More pronounced in vessels with smooth muscle regarding the intrinsic arm of the activation of factor muscles vWF & platelet adhesion coagulation pathway? XII, Elevated aPTT is primary hemostasis pa a. Begins with injury to the sub endothelium associated with an What substances are the principal ADP and Serotonin b. Elevated prothrombin time is associated abnormal function mediator of platelet aggregation? Bradykinin, fibrinopeptides, with abnormal function PT – for extrinsic a. ADP and Serotonin thromboxane a2, c. Begins with the activation of factor XII Injury to b. Bradykinin and fibrinopeptides endothelin – vascular d. Elevated aPTT is associated with an subendothelium – c. Calcium and Thromboxane A2 constriction abnormal function primary hemostasis d. Prostacyclin and Endothelin Calcium – Coagulation Which of the following statement/s is/are Which product in the circulation may serve TRUE regarding Heparin induced as a marker of thrombosis or other thrombocytopenia? condition in which a significant activation D-dimers a. Negative ELISA rules it out All of the choices are of the fibrinolytic system is present? Fibrin markers b. Platelet count drops by 50 percent from correct a. Adenosine 5-diphosphate FDPs, D-dimer, baseline b. Collagen soluble fibrin c. Positive ELISA does not confirm it c. D-dimers monomers d. All of the choices are correct d. Bradykinin Which rare autosomal recessive disorder Which of the following is an acquired Glanzmann Disseminated characterized by faulty platelet syndrome characterized by excessive thrombostenia intravascular aggregation and subsequent bleeding Bernard-Soulier thrombin generation and diffuse micro coagulation wherein glycoprotein Iib/IIIa is lacking or Failure of platelet adhesion thrombi formation? TTP – result of platelet dysfunctional? Von Willebrand a. Hemolytic uremic syndrome vWF is responsible for activation and A. von Willebrand’s disease carrying factor VIII and b. Disseminated intravascular coagulation formation of thrombi b. Hernansky – Pudlak syndrome platelet adhesion c. Thrombotic thrombocytopenic purpura c. Bernard – Soulier syndrome Hermansky d. Idiopathic thrombocytopenic purpura Loss of dense granules d. Glanzmann thrombostenia KUMUNOY’S IMPROPERTY 10  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. Which of the following statement/s is/are Which of the following statement/s is/are TRUE regarding Idiopathic TRUE regarding Tissue plasminogen thrombocytopenic purpura? activator? Selective for fibrin- a. In children it is usually acute in onset and All of the choices are a. Stabilizes the clot making it resistant to bound plasminogen short lived correct lysis Thrombomodulin b. In adults it has no identifiable cause b. All of the choices are correct yung thrombin sink c. All of the choices are correct c. Selective for fibrin-bound plasminogen d. It follows a viral illness in children d. Forms a complex with thrombin acting What protein is secreted by the alpha as a thrombin sink granules which serves to stabilize Which of the following statement/s is/are Thrombospondin fibrinogen binding to activated platelet TRUE for primary hemostasis? PF4 – promote blood surface and strengthens platelet to platelet d. Platelets initiate coagulation interactions? a release reaction recruiting other platelets vWF – carrying factor A. Thrombospondin in the circulation All of the choices are VIII and platelet b. Platelet factor 4 b. Platelet aggregation is reversible and correct adhesion c. Alpha thromboglobulin not associated with secretion d. von Willebrand factor c. All of the choices are correct What inhibitor of the fibrinolytic system acts Thrombin activatable d. Heparin does not interfere with these by removing lysine residues from fibrin? fibrinolysis inhibitor reaction a. Factor V Leiden “inhibitor of von Willebrand b. Thrombomodulin fibrinolytic system” factor Which protein is responsible for platelet c. Activated protein C daw eh so fibrinolysis natanong na kanina adhesion during injury to the sub d. Thrombin activatable fibrinolysis inhibitor inhibitor pero endothelium? Which of the following is a second line Rituximab P-selectin rolling a. Glycoprotein VI treatment for Idiopathic thrombocytopenic 1st: corticosteroid, IV or GPVI activation and anti-D immunoglobulin b. P Selectin purpura? aggregation of plt 2nd: splenectomy, c. von Willebrand factor a. Anti D immunoglobulin rituximab, thrombopoietin GPIIB/IIIA regulation d. Glycoprotein Iib/IIIa b. Rituximab 3rd: thrombopoietin of platelet adhesion c. Intravenous immunoglobulin receptor agonist, and aggregation combination Which of the following statement is TRUE Begins with the d. Corticosteroid chemotherapy regarding the intrinsic arm of the activation of factor Which of the following complex is 50 times coagulation pathway? XII more effective at catalyzing factor X a. Begins with the activation of factor XII Natanong na din activation? b. Begins with injury to the sub endothelium pero a. VIIIa-Ixa VIIIa-Ixa c. Elevated prothrombin time is associated PT – for extrinsic b. Activated protein C with abnormal function Injury to c. Prothrombinase d. Elevated aPTT is associated with an subendothelium – d. VIIa abnormal function primary hemostasis Dense granule What type of polymerization does removal deficiency What is the most common intrinsic platelet of fibrinopeptide A allow? most common ay defect? a. Side to side storage pool disease End to end a. Glanzmann thrombostenia b. Fibrinolysis (loss of dense b. von Willebrand’s disease c. End to end granules) c. Dense granule deficiency d. End to Side vWD – most common d. Bernard – Soulier syndrome Which glycoprotein on the platelet congenital bleeding membrane does von Willebrand factor disorder binds to? I/IX/V What is the required cofactor during the a. P selectin b. I/IX/V second wave of platelet aggregation that c. VI d. Iib/IIIa acts as a bridge between Glycoprotein Fibrinogen What is the initiating factor for acute Iib/IIIa and platelets? vWF – carrying factor coagulopathy of trauma (AcoT)? a. Fibrinogen VIII and platelet Shock a. Hypothermia b. Adenosine 5-diphosphate adhesion Shock and tissue b. Acidosis c. von Willebrand factor injury c. Dilution of coagulation factors d. Calcium d. Shock KUMUNOY’S IMPROPERTY 11  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. Removal of fibrinopeptide A will allow Which of the following is an acquired which of the following process in clot syndrome characterized by systemic formation? activation of coagulation pathways End to end a. Clot retraction resulting to excessive thrombin generation Disseminated polymerization b. All of the choices are correct and micro thrombi formation? intravascular c. End to end polymerization A. Disseminated intravascular coagulation coagulation d. Side to side polymerization b. von Willebrand disease Which of the following complication can c. Thrombotic thrombocytopenic purpura occur with rapid infusion of blood, plasma Transfusion- d. Idiopathic thrombocytopenic purpura expanders, and crystalloid particularly in associated Which of the following product in the older patients with underlying heart circulatory overload circulation may serve as a marker of disease? TACO hindi TRALI kasi thrombosis or other condition in which a D- dimers A. Hemolytic transfusion reaction yung symptoms na significant activation of the fibrinolytic Fibrin markers b. Disseminated intravascular coagulation nabanggit related sa system is present? FDPs, D-dimer, c. Transfusion-associated circulatory blood and heart A. Collagen soluble fibrin overload hindi naman lungs b. Bradykinin monomers d. Transfusion-related acute lung injury c. ADP Which of the following statement is/are true d. D- dimers regarding platelet concentrates? Escherichia coli a. Platelet concentrates can not transfuse Which of the following statements is/are infection infectious disease Shelf life is 5 days TRUE regarding hemolytic uremic Or Shiga toxin b. All of the choices are correct syndrome? producing bacteria c. Shelf life is 5 days a. All of the choices are correct d. Non allergenic b. Renal symptoms is less frequent Neurologic What is a sign of adequate resuscitation? c. Commonly associated with neurologic symptoms less a. Blood pressure equals to 80/50 mmHg symptoms frequent, renal b. Urine output equals to 1ml/kg/hr d. Escherichia coli infection failure can be seen Urine output equals c. Heart rate equals to 120 beats per so baliktad to 1ml/kg/hr minute Which of the following statements is/are d. Central venous pressure equals to 40 true regarding damage control cmH2O resuscitation? Which of the following statements is/are a. Minimize crystalloid based resuscitation All of the choices are TRUE regarding factor XIII? b. Immediate release and administration of correct a. Associated with severe bleeding pre defined blood products All of the choices are diathesis c. All of the choices are correct correct b. Male to female ration is 1:1 d. Permissive hypotension c. Bleeding is typically delayed Plasmin d. All of the choices are correct Thrombin ADP and serotonin Regulating Which of the following is/are the principal Bradykinin, Which of this enzyme is responsible for clot hemostasis and mediator/s of platelet aggregation? fibrinopeptides, degradation? maintaining blood a. ADP and serotonin thromboxane a2, a. Plasmin coagulation b. Thromboxane A2 endothelin – vascular b. Thrombin Thromboxane A2 c. Calcium constriction c. Thromboxane A2 Vasoconstriction d. All of the choices are correct Calcium – d. Fibrin Fibrin Coagulation Blood clot Which of the following can cause a patient Decrease absorption contraction or with liver disease to have a of vitamin K retraction hypocoagulable state? A and C – Which of the following is/are true regarding hypercoagulable a. Decrease production of protein C and S the extrinsic arm? b. All of the choices are correct Hypocoagulable – a. Measured by activated partial Initiated by vascular c. Decrease anti thrombin and decreased production of thromboplastin time injury plasminogen coagulation factors and b. Begins with the activation of factor XII A and B – intrinsic decreased absorption of c. Initiated by vascular injury d. Decrease absorption of vitamin K vitamin K d. All of the above KUMUNOY’S IMPROPERTY 12  BASIC SURGERY, TAHI-TAHI GUSTO KO, HINDI GANITO LECTURER: DOC BERNAL, BERY GOOD KA PO KUNG MAG-S’SAMPLEX KA. Which of the following condition is Which of the following is not related as a Hemolytic uremic confirmed by the presence of schistocytes blood transfusion complication? syndrome in the blood smear? Thrombotic a. Transfusion-related acute lung injury Other choices may a. Disseminated intravascular coagulation thrombocytopenic b. Transfusion-associated circulatory word na b. Thrombotic thrombocytopenic purpura purpura overload “transfusion” so alam c. Hemolytic uremic syndrome c. ABO hemolytic transfusion reaction na d. Idiopathic thrombocytopenic purpura d. Hemolytic uremic syndrome Which of following statements is/are true for Decreased 2,3 DPG banked whole blood? Ayoko masyado galawin ‘to, ‘di ako medtech HAHAHA, A – platelet a. Shelf life is 120 hours sabihin sa mga kumunoy if may debatable na samplex ha. concentrate b. All of the choices are correct C – indication for MAHIRAP ‘YUNG WOUND HEALING, GUMALING NA ‘YUNG c. Increases oxygen carrying capacity replacement SUGAT KO LAHAT-LAHAT, BAKA ‘DI PA RIN NAMIN TAPOS d. Decreased 2,3 DPG Which of the following characteristics of TRANS HAHAHAHA ABANGAN NA LANG. febrile non hemolytic transfusion reaction is a fairly common condition after transfusion? a. Increased in temperature more than 1 All of the above degree centigrade b. Acetaminophen reduces the severity c. It can be reduced by leukocyte washed RBCs d. All of the above Which of the following can be used to treat patients suffering from polycythemia vera? a. Low dose aspirin All of the choices are b. Hydroxyurea correct c. Phlebotomy d. All of the choices are correct Which of the following statement/s is true regarding heparin induced thrombocytopenia? a. Diagnosis maybe made by SRA/ELISA All of the choices are b. Platelet count typically falls 5 – 7 days correct after exposure c. All of the choices are correct d. Platelet count falls 1 – 2 days during re exposure Glanzman Which of the following is a rare platelet thrombasthenia genetic disorder that affects Glycoprotein Bernard-Soulier Iib/IIIa receptor resulting to faulty platelet Failure of platelet aggregation? adhesion Von Willebrand a. Idiopathic thrombocytopenic purpura vWF is responsible for b. Glanzman thrombasthenia carrying factor VIII and c. Hermansky – Pudlak syndrome platelet adhesion d. Bernard/ Soulier syndrome Hermansky Loss of dense granules Which of the following acts as a “thrombin sink” forming a complex with thrombin rendering it no longer available to cleave fibrinogen? Thrombomodulin a. Tissue factor pathway inhibitor b. Tissue plasminogen activator c. Thrombomodulin d. Urokinase-type plasminogen activator KUMUNOY’S IMPROPERTY 13