Test 1 Study Guide.docx

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Marist College
medical technology
Catherine Newkirk

Hematology Test 1 Study Guide Hemostasis- preventing hemorrhage (coagulation) 1. Three components: a. Extravascular- outside blood vessels (tissues) i. Tissues contract and release TF3 (tissue thromboplastin) ii. Tissue thromboplastin activates extravascular (extrinsic) pathway b. Vascular- maintain integrity= intact epithelium i. Platelets don’t stick factors inactivate (prostacyclin and coag factors) ii. Vasoconstriction- to prevent blood loss iii. Collagen exposed- activates platelets iv. Thromboxan A - wh2n endothelium damages also activates platelets and coagulation factors c. Intravascular- i. platelets form platelet plug (1: hemostatic plug) ii. release PF3 initiates intrinsic coagulation iii. Coagulation factors form fibrin clot around platelet plug 2. Bleeding Problems- w/ any of 3 components a. Weak vasculature- vessels b. Decreased number and/or function of platelets c. Decreased number and/or function of coagulation factors 3. Drawing- a. Basement membrane- (collagen) stimulates platelet aggregation (platelets sticking to other platelets) and adhesion (to something other than platelets- like collagen) b. Endothelial Cells- i. Prostacyclin- outside- potent platelet inhibitor- why platelets flow by and not activated ii. Thromboxan A2- inside- collagen exposed and platelets adhere 1. Stimulates platelet aggregation and adhesion 4. Platelets- (thrombocytes) a. 150,000-450,000/uL b. Young cell= the smallest (unique) c. Repeated nuclear division without cellular division (megakaryocyte) d. End product= platelet- cytoplasmic fragment- not really a cell e. Mature in bone marrow (<1% of all nucleated cells in BM) 5. Maturation Sequence- a. Megakaryoblast b. Promegakaryocyte c. Megakaryocyte- 32 to 64 nuclei d. Platelets- in peripheral blood i. 2-4uM in diameter ii. 2/3 in blood- 1/3 in spleen (slow transit) iii. Functions- 1. Maintain vascular integrity- keep blood inside vessels 2. Form platelet plugs upon activation (1: hemostatic plug) 6. Platelets Contain- a. Thrombasthenin- contractile protein b. ATP- energy c. Glycogen- energy d. Serotonin- vasoconstrictor- prevents blood loss e. ADP- platelet aggregation f. Epinephrine- platelet aggregation g. Ca - coagulation and platelet reactions h. Enzymes- viscous metamorphosis- mold into one platelet i. PF3- phospholipid initiates intrinsic coagulation j. PF4- fibrin monomer polymerization (form clot) and heparin neutralization i. Anti-thrombin agent (procoagulation) k. Clotting Factors- absorbed from plasma and picked up along the way 7. Platelet Plug- (when platelets are activated) a. Adhesiveness- collagen/CT/vWF  enables them to stick together i. Collagen- GPIb= glycoprotein Ib bridge b. Exogenous ADP- 1⁰ aggregation- other platelets sticking to ones already stuck (in plasma) c. Shape Change- swell then spikey d. Release Reaction- i. Ca++- coagulation and platelet aggregation ii. ADP- 2: aggregation- even more platelets stick iii. Serotonin- vasoconstrictor iv. PF3- intrinsic coagulation v. Enzymes- viscous metamorphosis- platelets fuse- platelet plug e. Clot Retraction- fibrin constricts due to thrombathenin= tight hard clot f. 1⁰ plug- not permanent until fibrin clot 8. Platelets Tested For- a. Number- (150,000-450,000/uL) i. Thrombocytopenia- 1. Abs destroy 2. Decreased production in bone marrow 3. Increased sequestering in spleen- splenomegaly 4. Increased destruction- heparin induced thrombocytopenia ii. Thrombocytosis/Thrombocythemia- (>500,000/uL) 1. Can’t predict function- can hemorrhage or throw clots all over body b. Function- thrombasthemia- abnormal platelet function 9. Tests- a. Platelet count- always do first no matter what i. Unopette- manual diluent on a hemacytometer ii. Slide Estimation- on scope count 10 even fields- take average and multiply by 20,000 iii. Automation- Coulter Counter b. Bleeding Time- (normal 4-6minutes) i. Use blood pressure cuff and pump to 40mmHg ii. Make incision and blot iii. Platelets adhere then aggregate iv. Capillaries contract v. **Testing for platelet and vascular number and function c. Aggregation – see #12 and picture d. Clot Retraction- red top-wait 24 hours i. In 24 hours tough/elastic- serum extracted ii. Dependent on thromboasthenin- do you have it? Is it functioning? iii. Glanzmanns- missing thrombasthenin iv. **Testing for platelet number 1. Thrombocytopenia would interfere with test e. Tourniquet Test- Rumple Leede i. Check for baseline petichiae ii. Increased blood pressure causes separation of endothelial cells iii. Platelets will fill in pores and blood vesesls won’t cause petichiae iv. Use blood pressure cuff and pump from 70-90 mmHg for 5 minutes v. >10 new petichiae= abnormal vi. **Testing for capillary fragility and/or function of platelets 10. Platelet Aggregation a. Platelet rich plasma added to aggregating agent- see below- short, low spin b. As platelets aggregate and fall out of solution- amount of light transmitted ↑ c. Agents- collagen, ADP, epinephrine, thrombin, arachidonic acid, ristocetin i. Arachidonic acid- exposes IIbIIIa glycoprotein on platelet and causes aggregation ii. Ristocetin- used for Von Willebrand’s – different mechanism see later picture d. Add Agents- i. Platelets swell and undergo primary aggregation (exogenous agent) ii. Release reaction- 2: aggregation (epinephrine, ADP, etc.) iii. Viscous metamorphosis iv. Platelet plug 11. Platelet Adhesiveness- (retention)- a lot of variability in these tests a. Ex Vivo- i. Platelet count ii. Swirl with glass beads- see how many platelets stick to glass beads iii. Recount platelets b. In Vivo- i. Serial plt counts from finger stick or bleeding time stick 1. See how decreases over time 12. Abnormal Vascular Function- no specific test a. Blood vessel and/or platelet function is problem b. ↑ bleeding time and tourniquet test (+) but aggregation and adhesion are finediagnosis of exclusion 13. Anti-Coagulant- a. Blue top- sodium citrate (removes Ca++)- 1:9 ratio (anti-coag:blood) b. Spin down- platelets in buffy coat c. 2 syringe technique bests- to avoid tissue thromboplastin contamination d. 6-7 mLs first 14. APTT Regent- activated partial thromboplastin time a. Add XII activator (kaolin or ellagic acid) b. Add PF3 substitute (cephalin) c. Incubate 37:c then add CaCl d. **Tests all but III, IV (Ca++), VII, XIII (not plug waiting) and PF3 (b/c add substitute) 15. Thrombin Time- a. Normal= 15 seconds b. **Testing for I (fibrinogen) 16. Coagulation Sequence- 17. Platelet Aggregation- 18. Plasma Coagulation Factors and Synonyms- Factor Synonym Function Factor I Fibrinogen Adhesive protein that forms fibrin clot Factor II Prothrombin Activated form is main enzyme of coagulation Factor III Tissue Thromboplastin Lipoprotein initiator of extrinsic pathway Factor IV Calcium Ions Metal cation necessary for coagulation Factor V Labile Factor, Proaceterin Cofactor for activation of prothrombin to thrombin Factor VII Serum Prothrombin Conversion Accelerator (SPCA); w/ tissue thromboplasin, initiates the extrinsic pathway stabile prothrombin conversion factor; proconvertin Factor VIII Antihemophilic globulin (AHG); anti-hemophilic Cofactor for intrinsic activation of Factor X factor (AHF) Factor IX Plasma thromboplastin component (PTC); Activated form is enzyme for intrinsic activation of Factor X Christmas Factor Factor X Stuart-Prower Factor Activated form is enzyme for final common PW activation of prothrombin Factor XI Plasma Thromoboplastin Antecedent (PTA) Activated form is intrinsic activator of Factor IX Factor XII Haegman Factor; Contact Factor Factor that normally starts APTT- based intrinsic pathway Factor XIII Fibrin Stabilizing Factor Transamidase that crosslinks fibrin clot HWM Cofactor- circulates in a complex with Factor XI Kininogen (Fitzgerald) Prekallikren Activated form that participates at beginning of APTT- based intrinsic (Fletcher) pathway Vitamin K dependent Lecture 2- Hemostasis 19. Factors a. Fibrinogen- i. Upon clotting is removed from serum (used up) ii. Precursor to fibrin- soluble globulin- produced in liver iii. 250-400 mg/dL= normal iv. Congenital and acquired deficiencies b. Prothrombin- i. Inactive zymogen (precursor) of thrombin ii. Vitamin K dependent to make gammacarboxyglutamic acid 1. Gammacarboxyglutamic acid- acts as a Ca++ binding site- without binding site is nonfunctional- PIVKA (protein induced by VitaminK antagonist/absenc) iii. Prothrombinase Complex- V- is cofactor which accelerates Xa and X3 (enzyme) iv. Congenital and acquired deficiencies c. Tissue Thromboplastin- i. Found in plasma membranes of endothelial cells ii. Not normally in circulation- unless injured iii. Extrinsic complex d. Ca++- i. low Ca++ levels cause tetany (muscle contractions and die before clotting problems) e. Labile Factor- aka proaccerlerin i. Deteriorates rapidly in plasma ii. Accelerates activity of Xa X3 f. Stabile Factor- aka proconvertin aka serum prothombin conversion accelerator (SPCA) i. Produced in liver ii. VitK dependent iii. Proteolytic enzyme in extrinsic complex iv. Congenital and acquired deficiencies g. Anti-hemophilic Factor/Globulin- (AHF/AHG) i. Complex glycoprotein w/ 2 distinct components: 1. Factor VIIIc- a. enters into coag for a clot- procoagulant portion b. Synthesis controlled by X chromosome- sex linked c. Activation of factor X through intrinsic complex d. Synthesized in liver e. Hemophilia A: (↓ VIIIc) i. Sex linked recessive deficiency ii. Bleeding excessively into joints and tissues (hemoarthrosis)= painful iii. Very short ½ life- must be injected- only give to bleeding patients iv. Genetically recombinate factor VIIIc 2. Factor XIII:Vwf a. Part of molecule involved in platelet adhesion “bridge”- labile b. Necessary for ristocetin aggregation c. Autosomal dominant gene (controlled by) d. Produced by platelets, megakaryocytes and endothelial cells e. Need to activate VIIIc h. Christmas Factor- aka plasma thromboplastin component i. Produced in liver ii. Vitamin K dependent iii. Activated by factor XIa iv. Needs Ca++ for activation v. Intrinsic complex (IXa) vi. Enzymatic activity vii. Sex-linked recessive- x chromosome viii. Hemophilia B- (if IX missing) 1. Clinically similar to Hemophilia A- treatment is different i. Stuart-Prower Factor- i. Rare hereditary and acquired deficiencies ii. Produced in liver iii. Vitamin K dependent iv. Prothrombinase complex v. Activated by intrinsic or extrinsic pathways j. Plasma Thromboplastin Anticedent- i. Activated XI + Ca++ causes activation of factor IX ii. All interact with each other: 1. Prekalekrin kalekrin (Fletcher Factor) 2. High molecular weight kininogen (Fitzgerald Factor) 3. Activated factor XII k. Hageman Factor- aka contact factor i. Ex vivo- silica tubes can activate ii. In vivo- collagen or PF3 can activate l. Fibrin Stabilizing Factor- don’t wait for in testing i. Acts w/ Ca++ to stabilize soft clots (fibrin(s) fibrin (I)) ii. Crosslinks fibrin polymers iii. Deficiencies= poor wound healing- umbilical cord never heals iv. Ca + Xa + IIa  XIII v. Fibrinolysis- 1. Delicate balance between clotting and bleeding 2. Xyminogen (precursor)= plasminogen (inactive) 3. Active enzyme= plasmin (dissolves clots)- BD fibrin and fibrinogen to FDP (fibrin degradation products) 4. MPS clears FDP 20. Anti-coagulants- a. Hypercoaguable- increased clot formation in some people i. Put on anti-coag for life (oral) b. Coumadin- aka warfarin, coumarin, dicumerol- all the same i. Derivatives of fermented sweet clover- in rat poison ii. Impairs ability of liver to use VitK (effects= II, VII, IX, X) iii. XII affected 1 - PIVKAS- no Ca++ binding site and don’t have GCGA on surface iv. Slow onsets (4-7 days)- long duration v. Monitor with PT b/c extrinsic and XII goes first c. Heparin- i. Immediate anti-coag- IV coagulant ii. Anti-thrombin activity iii. Short onset and duration (4 hours) iv. Monitor with APTT v. Protamine sulfate- neutralizes heparin (antecdote) vi. Anti-thrombin- (cofactor needed by heparin) 1. 1960’s- natural inhibitor of coagulation neutralizes thrombin (IIa)(as well as IX, X, VI, XII) and prevents clot formation 2. Heparin and antithrombin work instantaneously- alone they take longer 3. ↓ at birth= thrombotic events- young adults- stroke, heart attack a. Autosomal dominant gene- 1;2000-5000 have missing b. Synthesized in liver c. Treat with oral anti-coag d. Acquired form- liver disease, nephrotic syndrome (b/c it’s a small MW protein), surgical clotting, DIC, long-term anti-coag use Lecture #3- Review Testing 1. Tests for Vascular Integrity- a. Tourniquet test b. Bleeding time test 2. Tests for Platelets (number and function) a. Smear (platelet estimate and morphology) b. Platelet count c. Bleeding time d. Tourniquet test e. Platelet aggregation f. Clot retraction g. Platelet closure 3. Tests for Intravascular Coagulation Factors- a. Know vasculature if ↑ BT or tourniquet test but agglutination/adhesion tests normal- diagnosis of exclusion i. PT- (extrinsic and common PW) ii. APTT- (intrinsic and common PW) iii. Thrombin Time- (factor I-fibrinogen) iv. **Doesn’t test for III, IV (Ca++), XIII, PF3 4. Screening Tests- a. PT b. APTT c. Peripheral Smear- estimate and morphology d. BT- vasculature and platelets e. Platelet count- automated f. **If screening tests abnormal, the perform definitive tests g. ***most important screen= good medical history h. 1: hemostasis- (platelets and vasculature)- cutaneous and mucosal bleeding i. 2: hemostasis- (coagulation factors)- more serious bleeding 5. Definitive Tests- a. Prolonged BT- due to vascular disease, ↓ platelets, abnormal platelet function i. Consider vascular defects- (Vitamin C deficiency) ii. Assume platelet count and morphology were done on smear iii. Platelet aggregation- function iv. Platelet retention/adhesion- function v. Clot retraction- function vi. Tourniquet test- vascular and platelet function vii. Prothrombin consumption test- deficiencies in intrinsic PW including plts and Factor III b. Abnormal PT, APTT or both- i. Substitution studies ii. Factor assays iii. Thrombin time APTT PT Probably Deficiency N Abn Extrinsic, VII, substitution, corrected by russels viper venom Abn N Intrinsic, XII ,XI, IX, VIII, factor assays Abn Abn Common path or multiple factors, fibrinogen assay or thrombin time, II, VI, X substitution APTT  In addition to specific factor deficiencies, circulating anti-coags or specific factor Abs may prolong PT, APTT, or both o To distinguish from factor deficiencies:  10% Fresh normal plasma has all the factors and corrects all deficiencies  Inhibitors will be overcome gradually by an ↑ of fresh N plasma (~50%) o Prothrombin Consumption Test or Serum Prothrombin Time  Tests for PF3 and other intrinsic factors  Let blood clot in red top tube (uses I, II, V, VIII)  Remove serum (should contain VII, IX, X, XI and XII)  Perform PT (must add I and V*) o *BaSO4 absorbed normal plasma can be used (missing II, VII, IX, X- has I,V,VII,XI,XII)  Explanation- o The PT needs factors VII, X, V, II, I. Factor II should still be missing (used up in clot and not added back)  PT should be very long  If prothrombin (II) is present, there must be a problem with the initial clot in the red tube  Deficiencies in the intrinsic PW, including plts (qualitative or quantative-PF3), will cause prothrombin (II) to remain in the serum and the prothrombin consumption time will be shortened.  Factors VII and X effect the PT, therefore if the patient is deficient in either, the PCT will appear prolonged, which will be a false normal.  In other words…  Take serum from a red top tube (should be missing I, II, V, VIII)  Perform a PT after adding back factors I and V  Factor II should still be missing if original clot in red top tube is normal  The PCT- if normal- should be prolonged  If it is short- (~12-13 seconds), means factor II is present and therefore the original clot (in red top tube) was not normal  This test is not performed very often b/c only thing it tests for that the APTT doesn’t test for is PF3  It is a test in references labs and it helps one to thoroughly understand both the intrinsic and extrinsic clotting process.  o ISI= international sensitivity index o INR= 2-3 (most) o INR= 3-4.5 (mechanical valve/recurrent problems)  Protein S- o A vitamin K dependent plasma glycoprotein o Synthesized in the endothelium o In circulation exists in 2 forms: a free form (what we care about) and a complexed form with C4b- binding protein. o Inhibits blood clotting by serving as a cofactor for activated protein-C o Enhance fibrinolysis  Protein-C o A vitamin K dependent plasma protein o When activated by thrombin, inhibits the clotting cascade by enzymatic cleavage of factors V and VIII o Also enhances fibrinolysis o Deficiency results in recurrent venous thrombosis o When activated, complexes with S- together they inactivate V and VIIIc- no more thrombin production= no more coagulation  Both are autosomal dominant inherited genes  Synthesized in liver  Deficiencies-  Homozygous deficiency=fatal  Heterozygous deficiency= severe recurrent thrombosis o S and C activate plasminogen (dissolves clot)- fibrinolysis activated  Acquired Deficiency- liver disease, Vit K Deficiency, DIC (use it up), o Given long term anti-coag o Coumadin and Heparin to start-  If Coumadin given only to the pts- microembolism and skin necrosis b/c takes week to kick in so protein C ↓ faster than factorso II, VII, IX, X  Prevention of clotting goes faster than clotting so clots all over c. Anti-Thrombin- (Thrombin neutralization)- o Testing function o Whole venous blood- allow to clot o Let stand ~2 hours so can have anti-thrombin activity progress over time o Activated by prothrombin (II) o When start to clot, activate anti-thrombin- centrifuge and take serum (should have anti-thrombin) o Mix with normal plasma and thrombin (IIa) o ~15 seconds  Timing-  Normal with anti-thrombin= 33 +/- 3 seconds  Normal without anti-thrombin= <26 seconds d. Immuno-Rocket EP-  Electrophoresis in agar with immunodiffusion  Add Ab to anti-thrombin and do electrophoresis  If have thrombin will see rocket-like immunoprecipitate  Length tells you how much anti-thrombin is present  Testing quantity  Use controls e. ELISA-  Protein S and C- get quantity not function  40% of protei o n S= free (cofactor for C- acts in anti-coagulation- what we’re testing for)  60% of protein S= bound/complexed with C’ inhibitor of C’ pathway(C’ cascade) f. PCT- (platelet closure time)  Doesn’t test for vasculature  Replacing B tests for plt activity  Simulates vascular injury  Probes/membranes have collagen (exposure), epinephrine, or addinine-5-phosphate on them  Platelet stimulators filter plt rich plasma through membranes  Go to aperture (opening)  Should adhere (b/c activated) to aperture and eventually its occluded/ clogged  Report in seconds Lecture #4- Coagulation Disorders Factors Missing= Serum- I,II, V, VIII BaSO4- II, VII, IX, X 1. Hemophilia A- (Classic Hemophilia) a. One of the oldest known diseases; known since 5 century b. ~85% of hemophiliacs – 1/25,000 ppl effected c. Genetics: i. Factor VIIIc Deficiency ii. Synthesized in liver iii. Sex-linked recessive gene iv. Homozygous= not compatible with life v. Carrier female= (heterozygous)- varying levels of Factor VIII (normal excessive bleeding) vi. Before surgery must have >35%- give factor VIII concentrate vii. Abs can form to factor VIIIc- so only when bleeding are they treated 1. Immunosuppressant’s have little effect on Ab viii. After bleeding episode microcytic hypochromic anemia (over time) (↓Fe) 1. Leukocytosis 2. Reticulocytosis d. Severity- (important to test for levels) i. 0-5%=severe ii. 5-10%= moderate iii. 10-25%= mild e. Treatment- i. Factor VIII concentrate 1. ½ life of 12-18 hours, so can’t give prophylactically 2. FFP- put into circulatory overload 3. Cryoprecipitate- has I, VIII, VIII vWF, VIIIc a. Centrifuge at 4:c and pipet on ppt i. If have Abs to VIII concentrate give activated prothrombin complex (IIa-thrombin) 1. Dangerous b/c could cause clot anywhere f. Lab- i. BT= N ii. APTT= ↑ if = 20% Factor VIIIc iii. PT= N iv. PCT= Abn Short g. Clinical Symptoms- i. Crippling hemearthrosis- bleeding into joints ii. Bone changes- BM constantly producing new cells iii. Intracerebral bleeding iv. Intramuscular bleeding v. GI bleeding 2. Hemophilia B- “Christmas Disease” a. Genetics- i. Congenital- 1/100,000 people – 10% of hemophiliacs ii. Liver and Vitamin K dependent (IX) iii. Factor IX deficiency iv. Sex-linked recessive gene v. Doesn’t correspond w/ levels; don’t do level tests vi. Before surgery ensure have 30-40% factor IX b. To Differ from Hemophilia A- i. Substitution study- Will correct?  BaSO 4 Serum Hemophilia A Yes No Hemophilia B No Yes c. Lab- i. Resembles Hemophilia A clinically ii. APTT= ↑ iii. TT= N iv. PT=N v. BT=N vi. PCT= abN short- doesn’t use up I, II, V, VIII d. Treatment- i. Factor IX concentrate 1. 9 more stable than 8 (longer ½ life) 2. Given as a concentrate which contains 2,7,9.10 “prothrombin complex concentrate” aka proplex (not activated) ii. Normal 10% plasma iii. Serum iv. Cold Compress to ↓ vasculature 3. Congenital Factor V Deficiency- “Owren’s Disease” a. Genetics- i. Factor V deficiency ii. Autosomal recessive- 1/1,000,000 iii. Mild bleeding problems involving mucous membranes iv. Only need 10-15% Factor V to clot b. Lab- i. BT= N ii. APTT= ↑ iii. PT=↑ iv. TT= N v. PCT= abN short- not using I,II, V, VIII if original clot is normal FIRST QUESTION TO ASK! c. Treatment- i. 10% normal plasma ii. BaSO 4 iii. Fresh frozen plasma b/c it’s the labile factor 4. Congenital Factor VII Deficiency- a. Genetics- i. Autosomal recessive- 1/500,000 ii. GI/mucosal bleeding iii.Usually mild iv. Qualitative or quantitative deficiencies v. VII- synthesized in liver, VitK dependent vi. Associated with Dubin-Johnson Syndrome 1. Hereditary disorder of bilirubin metabolism (retain conjugated bilirubin in liver) b. Lab- i. APTT= N ii. PT= ↑ - can use russel’s viper venom as substitute iii. BT= N iv. TT= N v. PCT= abN long (↑) c. Treatment- i. Fresh frozen plasma ii. Proplex 5. Congenital Factor X Deficiency- “Stuart-Prower Deficiency” a. Genetics- i. Autosomal recessive- 1/100,000 people ii. Usually mild can be severe b. Lab- i. PT= ↑ (corrected w/: plasma, serum, celite 1. Celite= missing factor XI ii. PCT= abN Long iii. TT= N iv. BT= N c. Treatment- i. Proplex- contains II, VII, IX, X 6. Congenital Factor XI Deficiency- “Rosenthals Syndrome” or “Hemophilia C” a. Genetics- i. Autosomal recessive ii. 5% of hemophiliacs iii. Rare- 1/100,000 people iv. Heterozygous- mild- have 20% to 70% of normal XI- bleeding when surgery/trauma v. Homozygous- severe- have >15% of normal XI- bleeding problems w/o trauma vi. Shows up in Jewish population- not until an adult b. Lab- i. APTT= ↑- (corrected with: plasma, serum, BaSO4 ii. PT=N iii. BT= N iv. TT= N c. Treatment- i. Fresh frozen plasma 7. Congenital Factor XII Deficiency- “Hageman’s Disease” a. Genetics- i. Autosomal recessive ii. No symptoms in vivo b. Lab- i. APTT= ↑ in vitro- (corrected w/: plasma, serum, BaSO4, celite) ii. PCT= abN short c. History- i. Named after a railroad worker ii. Did Lee White clotting time and his took 120 minutes iii. Never had any bleeding problems iv. But died of clot to his lungs (pulmonary embolism) v. Ability of extrinsic system to bypass factor XII and activate the intrinsic system at Factor IX 1. Don’t have bleeding problems but have clotting problems b/c fibrinolysis is activated XIIa 8. Congenital Factor XIII Deficiency- a. Genetics- i. Autosomal recessive- rare ii. Shows up in infant unable to heal umbilical cord after birth- 36 hours after have bleeding episode iii. Poor wound healing iv. Plasma clots soluble in 5M urea (soft) b. Lab- i. All coagulation test= N ii. PCT= long 9. Congenital Fibrinogen Deficiency- a. Genetics- i. Autosomal recessive ii. 2 kinds: 1. Afibrinogenemia- rare a. Homozygous b. Severe 2. Hypofibrinogenmia- a. 20-100 mg/dL of fibrinogen (N= 200-400 mg/dL) b. Have abnormalities dependingon how much they make which effects lab tests accordingly iii. Lab- 1. All tests depending on fibrin clot= abnormal 2. Afibrinogenemia- a. All ↑ in tests looking for fibrin clot- except BT= N i. Corrected w/- plasma, BaSO4, celite 3. Hypofibrinogenemia- a. PCT= abN long (b/cadded factor I and V) 10. Congenital Prothrombin Deficiency- “Factor II Deficiency” a. Genetics- i. Rare ii. Autosomal recessive iii. Usually mild but can be severe b. Lab- i. PT= moderately ↑ - (corrected w/ plasma and celite) ii. APTT = moderately ↑ - (corrected w/ plasma and celite) iii. PCT= abN long 11. VonWillebrand’s- a. Genetics- i. Autosomal dominant ii. Effects both sexes equally iii. 1/10,000 people iv. On chromosome 12 v. Heterozygous= usually mild- doesn’t appear until 2 decade of life vi. Homozygous= moderately severe- presents early in life- gets better w/ age vii. Factor VIII glycoprotein 1. VIIIc= procoagulant 2. VIIIvWF- vonwillebrand’s factor a. Synthesis by endothelial cells, megakaryocytes and platelets b. Activated VIIIc in plasma c. Acts as bridge when plts attach to collagen- plt adhesion d. Acts as bridge for ristocetin aggregation i. Ristocetin not used as antibiotic anymore – only used when testing for VwF e. Low Factor VIII levels i. ↓ VIII activity b/c VIIIc not activated ii. ↓ VIIIvWF viii. Abnormal platelet function b. Symptoms- i. Bruising ii. Epistasis iii. Gingival bleeding iv. Prolonged menstrual period v. Excessive bleeding w/ tooth extractions vi. GI bleeding vii. Menorrhagia c. Lab- i. BT= ↑- (b/c platelets won’t stick to collagen) ii. ↓ Platelet adhesiveness iii. APTT= ↑ iv. PCT= abN short v. ↓ aggregation w/ ristocetin- normal w/ all others (correct w/: plasma, BaSO4) d. Treatment- i. Cryoprecipitate- contains factors I, VIIIc, VIIIvWF, XIIII ii. Fresh frozen plasma- contains all factors iii. DDAVP- deamino-8-D-arginine-vasopressin causes ↑ synthesis of VIIIvWF 12. DIC- (disseminated intravascular coagulation)- formerly “Consumption Coagulapathy” a. Genetics-
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