robbypowell's version from 2016-09-20 00:22

bleeding disorders

Question Answer
term: maintenance of clot-free, flowing blood within the vascular system, while also allowing the rapid formation of a solid clot to close ruptures/injuryHEMOSTASIS
term: the normal dissolution/breaking apart of fibrin clots; a normal component of hemostasisFIBRINOLYSIS
term for a pathologic process; the formation of a clot within the uninterrupted vascular system; a pathological extension of hemostasisTHROMBOSIS
term: a condition which makes the body tissues react with heightened susceptibilityDIATHESIS
term: any disorder of blood coagulation (bleeding or thrombosis)COAGULOPATHY
3 major contributing components of normal hemostasisVessels (esp. Endothelium), Platelets (aka Thrombocytes) & Coagulation Proteins
what vitamin deficiency causes bleeding problems by affecting blood vessels?Vit C (ascorbic acid)
What cell gives rise to Thrombocytes?Megakaryocyte
All coagulation proteins except for _____ are produced in the ___Von Wildebrandt factor; Liver
steps of hemostatic plug of vessel breachI-V-A-A-C: Injury (endothelium), Vaso-CONSTRICTION, Platelet Adhesion (to collagen), Activation, Coagulation cascade
What effect does uninjured endothelium have on blood coagulation?Anti-Coagulant effect (inhibiting)
What effect does injured endothelium have on blood coagulation?Pro-Coagulation effect (promoting)
___ is critical in platelet activation (ion)Calcium
Which coagulation factors are Vit K dependent?2, 7, 9 & 10
___ is critical in coagulation cascade (ion)Calcium
what are 2 sources of Vit K?Leafy Vegetables & Intestinal bacteria (E. Coli)
Vit K is ____ soluble and absorbed where?Fat soluble; Sm. Intestine
4 components of clotRBC's, PMN's (or other leukocytes), Platelets, & 5' Polymerized Fibrin
Platelet deficiencies lead to ____ bleedsSmall
Coagulation factor deficiencies lead to ____ bleedsLarge!
e.g. of hereditary bleeding disorder affecting blood vesselsHereditary hemorrhagic Tele-angi-ectasia
What is the receptor for Von Willebrand factor?Glycoprotein 1 beta (GP1b)
What is GP1b a receptor for?Von Willebrand factor (VWf)
How do platelets adhere to collagen (exposed during injury)1. VWf adheres to collagen & 2. GP1b adheres to VWf
What part of Platelet activation involves GP2b/3a & fibrinogen?Aggregation (sticking to each other)
What part of Platelet activation involves VWf & GP1b?Adhesion (to collagen) and Shape Change
After adhesion and shape change a platelet secretes ____ ___ & ____ ___Alpha Granules & Dense Bodies
What binds to GP2b/3a receptors?Fibrinogen (binds to these receptors on both ends forming bridge) (Aggregation)
T/F: Higher concentrations of Calcium in platelets leads to greater platelet activationTrue
Tissue damage can cause activation of the coagulation cascade by release of ____ which activates Coagulation Factor # ___... this is the ____ pathwayTissue Factor; #7, Extrinsic
Both the Intrinsic and Extrinsic pathways of the coagulation cascade are means to activate Coagulation Factor # ___10
The Common Pathway of Coag Cascade begins by activating CF # ___ and ends with formation of ____10; Fibrin
3 Infections (microbes) that can cause vascular disorders1. meningo-coccemia, 2. rickettsiae, 3. ebola
Endocrine disorder associated with vascular disordersCushing Syndrome
a connective tissue disorder, mutations in collagen etc. leading hypermobility of joints, increased bruisability, marked elasticity of skinEhlers-Danlos syndrome
autosomal dominant hereditary condition with dilated tortuous vessels with thin walls; most common manifestations are bleeding in mucosa of nose, mouth, eyes, GI tractHereditary hemorrhagic telangiectasia (aka Weber-Osler-Rendu syndrome)
Weber-Osler-Rendu syndrome is akaHereditary Hemorrhagic Tele-Angi-Ectasia
Hereditary Hemorrhagic Teleangiectastia is akaWeber-Osler-Rendu syndrome
Platelet disorders caused by reduced platelet count are called _____Thrombocyto-penias
Platelet disorders caused by defective platelet function are called ____Thrombocyto-pathies
Normal platelet count per microliternormal = 150,000-450,000 per microliter
Thrombocytopenia is defined as < ______ platelets per microliter<150,000
Signs/symptoms of Thrombocyto____: petechiae, purpura (small vessel) bleeding in skin, mucous membranes of oral cavity and other GI sites, GU tract (hematuria)Thrombocyto-PENIA (few platelets)
patients will have an Increased risk of intracranial bleeds with a platelet count under ...<10,000/mm3
patients will have Spontaneous bleeding with platelet count under...<20,000/mm3
T/F: Most Thrombocytopenias are acquiredTrue
T/F: Most Thrombocytopenias are hereditaryFalse (most are acquired)
3 types of "drugs" that can lead to Thrombocytopeniaalcohol, thiazides, cytotoxic drugs
2 infections that can lead to ThrombocytopeniaMeasles & HIV
What deficiency can lead to Thrombocytopenia?B12/Folate
Hyper plastic enlargement of what organ can cause Thrombocytopenia?Spleen (Splenomegaly)... (more of them are hanging out in spleen reservoir)
3 drugs that can lead to Thrombocytopenia by increasing destruction of platelets in the bloodsulfa, quinidine, heparin
Patient with acute thrombocytopenia of unknown cause... you should suspect what cause?Drug-induced (over 100 drugs can induce... including herbal remedies)
T/F: The vast majority of Thrombocytopathies are acquired/iatrogenicTrue
T/F: The vast majority of Thrombocytopathies are hereditaryFalse (acquired or iatrogenic)
Normal platelet count but prolonged bleeding time points to what category of platelet disorders?Thrombocytopathies (function, not #)
Two normal OTC drugs that can affect clotting ability (platelet function) for 10 daysAspirin & Ibuprofen
Test used to assess function of the Intrinsic Pathway (Contact Activation Pathway)Partial Thromboplastin Time (PTT)
Test used to assess function of Extrinsic PathwayProthrombin Time
Tests used to assess function of the Common PathwayPTT (Partial Thromboplastin Time) & PT (Prothrombin Time)
Group of conditions associated with Coagulation Factor deficienciesHemophilias
Normal platelet count; prolonged PT &/or PTTHemophilia (Coag Factor deficiencies)
Diseases of which organ are likely to affect multiple coagulation factors?Liver
What vitamin deficiency can lead to hemophilia?Vit K
T/F: The majority of Hemophilias are acquiredTrue
T/F: The majority of Hemophilias are hereditaryFalse (acquired)
T/F: Chronic Liver disease can lead a wide range of bleeding symptomsTRUE (b/c balance of different factors can be affected in a number of ways)
T/F: Chronic Liver disease can cause a narrow range of bleeding conditions, such as hemophiliaFALSE, (does cause hemophilia, but can cause Thrombosis too... apparently)
Hospitalized &/or severely debilitated patients can get multifocal thrombosis and then eventually break these up leading to hemorrrhage and infarcts.. this is a condition called...Disseminated Intravascular Coagulation
Hereditary Factor VIII deficiencyHemophilia A
Hereditary Factor IX deficiencyChristmas disease (Hemophilia B)
Most common hereditary HemophiliaVon Willebrand's disease
3 most common hereditary hemophilias from most to leastVW disease, Hemophilia A, & Hemophilia B (Christmas disease)
Which hereditary hemophilias are X-linked recessive?Hemophilia A & B (factors 8 & 9 respectively)
What hereditary hemophilia presents with signs and symptoms similar to platelet deficiency?VW disease
T/F: Hemophilia A and B are clinically indistinguishableTrue
___ are given orally and antagonize the action of vitamin K, so that the prothrombintime (PT) and activated partial thromboplastintimes (APTT) are prolongedCoumadins (e.g. Warfarin)
usual INR therapeutic range for deep vein thrombosis2-3
INR required for patients with prosthetic heart valves4.5
ratio of a patient's prothrombin time to a normal (control) sampleINR (International Normalized Ratio)
Which factors should be affected by Coumadin drugs? (warfarin e.g.)2, 7, 9 & 10
Cancer that can cause thrombotic disorders due to increased protein in circulationMultiple Myeloma
2 conditions that can cause thrombotic disorders due to increased productions of RBC'spolycythemia vera & COPD
cancer that can cause thrombotic disorder due to increased WBC's in circulationLeukemia
E.G. for cause of excessive platelet productionEssential Thrombocytosis


Question Answer
High von Willebrandfactor, low platelet counts... _____ hemostasisPrimary
Low anticoagulant factors (protein C, antithrombin) and low procoagulant factors (fibrinogen, II, V, VII, IX, X XI)Coagulation
Coag Profile: Normal platelet count; prolonged PTT; decreased Factor VIII activityHemophilia A (or B)


Question Answer
1-2mm (pin point) red-purple maculesPetechiae
2-5mm red-purple maculesPurpura
1-2cm red-purple maculesEcchymosis
swelling due to blood collecting in a space or creating a space between layers of tissueHematoma
chronic bleeding into joints leading to fibrosis and ankylosisHemarthrosis

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