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Hematology Congenital Coagulation Defects
Exam 6 Lecture 37 Rodak Chapter 41
92
Biology
Undergraduate 3
11/19/2014

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Term
Defects in Factors can be due to (4)
Definition
Decreased synthesis (Liver)
Production of abnormal molecules
Consumption
Inhibitors or ABYs
Term
Clotting factor defects may be _____ or ____
Definition
inherited
acquired
Term
Congenital defects may be ______ or ______ or _________
Definition
Sex-linked
Autosomal dominant
Autosomal recessive
Term
Plasma coagulation factors are cofactors or precursors of ____ ______ that circulate as inactive substances
Definition
serine proteases
Term
T or F:
Clot formation is the result of activation and the subsequent cascade
Definition
True
Term
T or F: If one factor is missing, blood will NOT clot
Definition
True
Term
Fibrinogen is an ______-____ ______
Definition
acute-phase reactant
Term
T or F: Physiologic stress can cause an increase in the fibrinogen produced
Definition
True
Term
A large glycoprotein produced in the liver
Definition
Factor I (Fibrinogen)
Term
Normal fibrinogen levels are
Definition
200-400 mg/dL
Term
Factor I Deficiency APTT and PT are
Definition
increased
Term
Afibrinogenemia
Definition
No fibrinogen
Term
Hypofibrinogenemia
Definition
Low fibrinogen
Term
Dysfibrinogenemia
Definition
Qualitative disorder
Fibrinogen is not working properly
Term
Factor I Deficiency treat with
Definition
FFP
Cryo
Term
FFP contains ___ ___
Definition
all factors
Term
Cryo contain ____, ____, ____
Definition
I
VIII
XIII
Term
Fibrinogen problems are usually _____ rather than ______
Definition
acquired
congenital
Term
Synthesized in the liver

Most abundant of the vitamin-K dependent clotting proteins
Definition
Factor II (Prothrombin)
Term
Factor II - _______ PT and APTT
Definition
Increased
Term
Deficiencies do not occur because it is found only in the tissue
Definition
Factor III (Thromboplastin)
Term
If too low to clot, incompatible with life
Definition
Calcium
Term
Acts as a cofactor in the activation of prothrombin

Produced in the liver
Definition
Factor V (Proaccelerin)
Term
Congenital factor V deficiency is also known as
Definition
parahemophilia
Term
Parahemophilia is an ____. ____-___ ____
Definition
inherited, autosomal-recessive trait
Term
T or F: Factor V Deficiency patients will have mild clinical disorders
Definition
True
Term
Factor V Deficiency - APTT and PT are _____
Definition
prolonged
Term
Treat Factor V Deficiency with
Definition
FFP
Term
FFP stands for
Definition
Fresh Frozen Plasma
Term
Vitamin-K dependent factor

Produced in the liver

Treat with FFP
Definition
Factor VII (Proconvertin)
Term
Factor VII (Proconvertin) is _____ as an ____ _____ deficiency
Definition
Inherited
Autosomal recessive
Term
Factor VII (Proconvertin)- ______ PT and _____ APTT
Definition
Prolonged
Normal
Term
A large glycoprotein
Made in the liver
Not Vitamin K dependent
Factor is labile
Definition
Factor VIII (Anti-Hemophiliac)
Term
T or F:
You will not find Factor VIII in serum
Definition
True
It will be consumed in the clot
Term
2 portions of the Factor VIII molecule
Definition
VIII: C
VIII: vWF
Term
Coagulant and smaller portion of Factor VIII
Definition
VIII: C
Term
Necessary for Plt adhesion to collagen
Definition
VIII: vWF
Term
Attaches collagen to glycoprotein IB on Plt surface
Definition
VIII: vWF
Term
Two diseases associated with factor VIII deficiency
Definition
Hemophilia A
Von Willebrand's disease
Term
Factor VIII factor assay will be _____ in both Hemophilia A and Von Willebrand's disease
Definition
decreased
Term
Deficiency is the clotting portion only; smaller protein unti
Definition
Hemophilia A
Term
Deficiency is the clotting portion as well as platelet adhesion; larger protein unit
Definition
Von Willebrand's disease
Term
The most common hereditary coagulation disorder
Definition
Hemophilia A
Term
T or F:
Hemophilia A is a sex-linked recessive bleeding disorder
Definition
True
Term
How much of Factor VIII normal is found in the following:
Severe
Moderate
Mild
Carrier
Definition
<1%
3-5%
5-12%
50% activity
Term
Severe bleeding into joints
15% will develop ABYs to the VIII factor
Definition
Hemophilia A
Term
Hemophilia A
_____ APTT
_____ PT
_____ Bleeding Time
Definition
Increased
Normal
Normal
Term
T or F:
With hemophilia A, APTT will correct with absorbed plasma but not serum
Definition
True
Term
Treatment for Hemophilia A (3)
Definition
Human VIII factor
Cryprecipitate obtained when thaw FFP at 1-6 degrees Celcius
Newer factor VIII concentrates are powdered
Term
Autosomal inherited disorder
prolonged bleeding times
Mucocutaneous bleeding (dentist), bruising
Definition
Von Willebrand's Disease
Term
Von Willebrand's Disease:
APTT will be ______
__ _ ___ VIII: vWF
FVIII:C activity will also be ______
Definition
Prolonged
Low or Absent
Abnormal
Term
T or F:
Von Willebrand Disease - Factor is stable
Definition
True
Term
Treat Von Willbrand Disease with
Definition
FFP
Cryo
Term
T or F:
Von Willebrand Disease - Abnormal platelet aggregation with ristocetin
Definition
True
Term
Is Von Willebrand Disease Dominant or Recessive?
Definition
Dominant
Term
Christmas Factor
Definition
Factor IX
Term
Factor IX: Present in ____ but not ___ ___
Definition
Serum
Absorbed Plasma
Term
Factor IX is acquired in ___ ___ or ___ ___
Definition
Liver Disease
Coumadin Therapy
Term
IF Factor IX Deficiency is inherited it is referred to as
Definition
Hemophilia B
Term
T or F:
Factor IX is a Vitamin K Dependent factor and is synthesized in the liver
Definition
True
Term
Treat Factor IX with
Definition
FFP
Cryo
Prothrombin Complex
Term
Factor IX
_____ APTT
_____ PT
Definition
Increased
Normal
Term
Factor IX will _____ with serum but not with ___ ____
Definition
correct
Absorbed plasma
Term
Stuart-Prower Factor
Definition
Factor X
Term
Deficiency is rarely inherited
Vitamin K Dependent
Produced in the liver
Definition
Factor X (S-P Factor)
Term
Most severely affected Factor X Deficient patients will have ___-____ ____
Definition
post-operative hemorrhage
Term
Factor X:
______ PT
______ APTT
Definition
prolonged
prolonged
Term
Plasma Thromboplastin Antecedent
Definition
Factor XI
Term
Inherited form is called Hemophilia C
Definition
Factor XI
Term
Hemophilia C is an ___ ___ trait
Definition
Autosomal recessive
Term
T or F:
Factor XI is one of the contact factor proteins involved in early coagulation and it is synthesized in the liver
Definition
True
Term
Factor XI:
____ PT
____ APTT
Definition
Normal
Abnormal
Term
Hageman Factor
Definition
Factor XII
Term
T or F:
Factor XII Disorder is NOT associated with clinical bleeding or hemorrhage
Definition
True
Term
Factor XII is _____ in an ___-___ fashion
Definition
inherited
autosomal-recessive
Term
With Factor XII there is an increased incidence of _____ ____
Definition
thrombotic disease
Term
Factor XII Deficient patients will be prone to having ___ ____ or _____
Definition
Myocardial infarction
thromboembolism
Term
Factor XII
_____ APTT
Definition
Abnormal
Term
T or F:
Is is difficult to monitor heparin in a patient with a Factor XII Deficiency
Definition
True
Term
Factor ____ is responsible for the formation of a stable fibrin clot
Definition
XIII
Term
Fibrin-Stabilizing Factor AKA
Definition
Factor XIII
Term
The only enzyme in the coagulation system that is not a serine protease
Definition
Factor XIII (F-S Factor)
Term
Factor XIII _____ with fibrinogen
Definition
Circulates
Term
With which factor deficiency does the following occur:
In homozygous cases, there is initial stoppage of bleeding followed by recurrence of bleeding 36 hours after the initial traumatic event
Definition
Factor XIII (Fibrin-Stabilizing Factor)
Term
Factor XIII deficiency - the bleeding is the result of the
Definition
dissolution of the fibrin clot that initially formed and was not stabilized by Factor XIII
Term
Factor XIII:
____ APTT
____ PT
____ BT
____ Plt Count
____ fibrinogen
Definition
All Normal
Term
Screening test for XIII is based on the ____ of a ____ ___ ___ in a 5-molar ___ ___
Definition
solubility
recalcified plasma clot
urea solution
Term
The screening test for Factor XIII deficiency is referred to as the
Definition
Urea solubility test
Term
Fletcher Factor
Definition
Prekallikrein
Term
Fitzgerald Factor
Definition
High-Molecular-Weight Kininogen
Term
Prekallikrein:
May experience ___ ___
____ ____ of the APTT
Definition
thrombotic events
Marked prolongation
Term
HMWK:
May experience ___ ___ ___ and ___ ___
____ ____ APTT
Definition
deep vein thrombosis
pulmonary embolus
Markedly prolonged
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