Coag Exam 2 Flashcards
Coagulation Proteins (Factors)
In circulation:
Activated Form:
Purpose:
In circulation: inactive (zymogen)
Activated Form: interact to form fibrin Clot
Purpose: to reinforce the plt plug
Factor 1
Fribrinogen
Factor II
Prothrombin
Factor III
Tissue Factor (tissue thromboplastin)
Factor IV
Ionized calcium
Factor V
Labile Factor (proaccelerin)
Factor VII
Stable Factor (Serum prothrombin conversion accelerator SPCA), proconvertin
Factor VIII
Antihemopholic factor, factor VIII:C (coagulation portion)
Factor IX
Christmas Factor, Plasma thromboplastin component (PTC), antihemophilic factor B
Factor X
Stuart-Prower factor
Factor XI
Plasma Thromboplastin Antecedent (PTA)
Factor XII
Hageman factor (contact factor)
Factor XIII
Fibrin-stabilizing factor (FSF)
Fitzgerald factor
High-Molecular Weight Kininogen (HMWK)
Fletcher factor
Prekallikrein
Vitamin K dependent
Factors: 2, 7, 9 and 10
Protein C and S
effects of Coumarin, Coumadin and Warfarin
- inhibit Vit K reduction yielding inactive factors
- only active in vivo (unlike heparin)
Fibrinogen or Thrombin sensitive proteins
1, 5, 8c and 13
Thrombin acts on all factors in the fibrinogen group
Enhances activity of:
Activates factor:
Converts:
Enhances activity of factors 5 and 8c
Activates factor 8
Converts fibrinogen to fibrin
PT is measured by which pathway?
Extrinsic
APTT is measured by which pathway?
Intrinsic
3 disorders of Fibrinogen
Afibrinogenemia, hypofibriogenemia and and dysfibrinogenemia
What is afibrinogenemia?
quantitative disorder
lack of synthesis in liver
What is hypofibrinogenemia?
low amounts of fibrinogen and is generally asymtomatic
What is dysfibrinogenemia?
abnormal fibrinogen structure and function
symptoms: post-traumatic or postoperative bleeding of mucosal tissues
What does factor 8 do?
functions as a catalyst, forming bonds between proteins (fibrin monomers, fibronectin, collagen, alpha 2-inhibitor)
What is factor 8 characterized by?
initial stoppage of bleeding then recurrence of bleeding more than 36 hours after event
Lab results and treatment for factor 8 deficiency
Normal: PT, APTT, TT, BT, fibrinogen, plt count
Low levels: F8 detected by 5M urea test
Treatment: FFP or cyro
3 acquired disorders that are secondary to other pathological events
DIC, primary fibrinolysis and liver disease
What is DIC?
disseminated idiopathic coagulation
Classification: consumption coagulopathy (plt and factors depleted)
Physiologic effects: plasminogen activated, results in increased FDP’s and possible RBC fragmentation
Lab results for DIC
Plt count: decreased
Fibrinogen: decreased
PT, APTT, and TT: prolonged
FDP or d Dimer: elevated
Cause of DIC
pathway activation
Extrinsic: large amounts of tissue factor entering blood stream
Intrinsic: events that damage vascular endothelium exposing collagen
Treatment for DIC
FFP, plts, cryo, low molecular weight heparin to break cycle and treat symptoms, remove stimulus
What is Primary Fibrinolysis?
symptomatically similar to DIC
results from increased levels of plasmin
Patient presentation: diffuse hemorrhages due to increased plasmin fibrinolytic activity and will initially form a clot that dissolves in 1-2 hours
Causes of primary fibrinolysis
cirrhosis, shock, metastatic CA of prostate, injury to urinary tract or leakage of urokinase from urine into tissues
Lab tests for fibrinolysis
Fibrinogen: decreased PT and APTT: prolonged FDP: increased d-Dimer: normal Plt count: normal RBCs: normal, no fragmentation
In liver disease a decrease in what factor happens first and why?
Factor 7 because it has the shortest half life
What is seen in 1/3 of chronic liver disease patients?
Thrombocytopenia due to spleen sequestration secondary to congestive spenomegaly
What is the most common hereditary coag disorder and what is it’s inheritance pattern?
Hemophilia A ( Factor 8:C)
X-linked recessive-factor 8:C on X
*males more effected
_________ or ________ develop in 10 to 15% of factor 8:C deficiencies.
alloantibodies or inhibitors
Symptoms of Hemophillia A?
Hemarthrosis Hematuria Intracranial bleeds Hematomas Spontaneous hemorrhage
Lab Findings
1-BT 2-PT and TT 3-APTT 4-Factor 8:C activity 5-vWF: Ag 6-Plt function assays 7-mixing studies
1-Normal 2-Normal 3-Prolonged-Factor 8:C level< 20% 4-Low to absent 5-Normal 6-Normal 7-mixing studies do not correct with pooled plasma
Treatment for Hemophilia A?
Replacement therapy or Cryo
1-Hemophilia B?
2-aka
3-inheritance pattern
4-activated by
1-Factor 9 deficiency
2-Christmas disease or Plasma thromboplastin component
3-sex linked- less common than Hemophilia A
4-XIa, Ca2+,via TF and Factor VIIa or Russell Viper Venom
Treatment Hemophilia B
- regular infusion with FFP (contains active F9)
- prothrombin complex ( F2,7,9,10)
- or F 9 concentrates
Hemophilia B Lab Tests
1-PTT,TT,BT
2-APTT
3-mixing studies
1-Normal
2-Increased
3-corrected with normal pool or aged serum
1-Hemophilia C
2-aka
3-found in what population
1- Factor 11 deficiency
2-Rosenthal syndrome
3-Ashkenazi jewish population
Is replacement therapy necessary?
no unless patient is schedule for surgery- then they need FFP to increase factor assay level to 20-30%
Factor 11 levels >120% at risk for ?
thrombosis
Hemophilia C Lab Tests
1-PT,BT,TT
2-APTT
3-Factor 11 assay
1-normal
2-Increased
3-decreased (normal 70-130%)
Factor 7 deficiency
1-A.K.A.
2-inheritance pattern
1-procoverntin
2-rare auto recessive
Symptoms of Factor 7 deficiency?
- deep muscle hepatomas
- joint hemorrhage
- epistaxis
- menorrhagia
Lab Tests for Factor VII Deficiency
1-APTT,TT,BT
2-PT
1-Normal
2-prolonged (fully corrected with Russells Viper venom (activates FX) and mixing with aged serum
Factor X Defeciency
1-AKA
2-inheritance pattern
1-Stewart Prower Factor
2-rare auto recessive at any age
Normal reference range for Factor X?
50-150%
Factor X lab test?
1-PT,APTT
2-TT,BT
3-Stypfentime or RVVT
1-abnormal
2-normal
3-prolonged
Treatment of Factor 10?
FFP or Prothrombin concentrates
Factor V
1-AKA 2-Catalyst in? 3-inheritance 4-normal range? 5-Acquired FV?
1-labile factor ( because of rapid deterioration in plasma at room temp
2-conversion of conversion of FII to IIa
3-Rare autosomal recessive
4-50-150%
5-specific Ab acquired after childbirth or use of fibrin glue in surgery (associated with liver disease, carcinoma, TB, DIC, etc…
Lab tests for Factor V deficiency?
1-specimens
2-APTT,PT
3-TT
4-
1-Specimens must be diluted (platelet poor)
2-prolonged
3-Normal
4-FFP
*not cry because there is not enough FV in concentrate
Factor II Deficiency
1-AKA
2-delays what?
1-prothrombin
2-delays generation of thrombin-hemorrhagic symptoms
What is the normal reference range for Factor II and i has the longest what?
- 50-150%
- has the longest 1/2 life of the Vitamin K factors
Factor II is converted to thrombin by what?
the action of Factor Xa and Va + Platelet Factor 3 + Ca2++ ( known as prothrombininase complex, assembled on plot surface
Factor II (Hypoprothrombinemia)
Lab tests
1-PT,APTT
2-TT
3-Diagnosis dependent on?
1-prolonged
2-Normal
3-….activity assay or antigenic concentration of prothrombin or both ( both should be decreased)
Factor II (Dysprothrombinemia)
structural defect that causes impaired activity
Treatment for Factor II issues
- FFP
- prothrombin complex factor concentrates
Factor II prothrombin mutations (G20210A). 1-occurs where? 2-2nd most common cause ? 3-restricted to what population? 4-Must be detected via? 5-identified as risk factors for
1-chromosome 11 2-of inherited thrombophilia 3-caucasion 4-DNA analysis by PCR 5-MI in young women, stroke in young patients
Hageman (Factor 12 deficiency)
1-aka
2-Lab tests (PT,APTT, F12 analysis)
3-normal reference range
1-Hageman trait 2-PT- Normal -APTT-prolonged -Factor 12 analysis-decreased 3-70-140%
Prekallikrein (Fletcher) deficiency
1-clinical bleeding?
2-prediposition to race?
3-APTT
4-contact activation time in APTT kaolin-like reagents changes?
1-no
2-none
3-marked prolonged
4-incubation time intervals will progressively shorter the APTT
