Day 3- hemophilias

symptoms of secondary hemostasis

• delayed bleeding

• deep muscle bleed

• spontaneous joint bleed

most common autosommal dominant bleeding disorder is

von Willebrand disease

Which secondary hemostasis are X-linked recessive

• F VIII deficiency

• F IX deficiency

What are the vitamin K factors

• Factors II, VII, IX, and X

• Protein S and C

• PIVKA

What factor can activate more factors VII in the extrinsic system

FXa

When FXII is activated by TF what forms

extrinsic tenase complex

Extrintrisic Tenase complex contains

• TF

• VIIa

• IX

• X

• on the surface of the plt

What does the extrinsic tenase complex do

• connect the intrinsic and extrinsic pathway

• It activates factor X, leading to thrombin generation and the propagation of the coagulation cascade.

FX when activated forms

Prothrombinase complex

Prothrombinase complex is composed of

• activated factor X (Xa) and prothrombin (II), along with factor V.

• creates thrombin

When a sample is left at room temp what happens in it

• FV deteriorates

• PF4 inactivates heparin and platelet function is impaired.

What is made from HMWK and causes vasodilation and smooth muscle cntraction

Bradykinin

HMWK and Bradykinin causes

inflammation

Prekalikrein and kallikrein causes

complement activaion

the combination of the below cause:

• XIa

• Plasminogen

• plasmin

• and kallikrein

fibrinolysis

The combination of XI HMWK create XIa which results in

intrinsic coagulation pathway

What activates FXII

contact with a negatively charged surface (glass)

deficiency in FXII causes

hyper clotting

What is the main job of VIII

acceleration of factor IXa and X

What inactivates FVIII

Protein C

In the formation of Fibrin what gets cleaved off fibrinogen and what cleaves them?

• fibrinopeptides A and B

• thrombin cleaves them

what causes aquired FVII deficiency

• liver disease

• coumadin/wafarin

• Broad spectrum antibiotic use

in FVII deficiency testing shows

• plt count normal

• bleeding time normal

• PT increased

• PTT normal

deficiencies in contact factors FXII, HMWK or prekallikrein show what symptoms

none its asymptomatic usually

deficiencies in contact factors FXII, HMWK or prekallikrein show what tests

• Plt count normal

• Bleeding time Normal

• PT normal

• PTT longer

Hemophilia A has what factor deficiency

F VIII

Hemophilia B has a deficiency in factor

F IX

Hemophilia C has a deficiency in

F XI

Heophillia C is seen in what population

Ashkenazi jew

testing if XI deficiency includes

• Plt count normal

• bleeding time normal

• PT normal

• PTT would be long

Testing for F IX deficiency includes

• Normal platelet count

• Normal bleeding time

• Normal PT

• Prolonged PTT

Factor VIII and VWF are

married and circulate together

Hemophilia A has what symptoms

bleeding into muscles, soft tissues and joints

Complications with hemophilias include

neutralizing antibodies

What tests would show FVIII deficiency

Plt count normal

bleeding time normal

PT normal

PTT long

VW disease type 1

partial quantitative deficiency in vWF

VW disease type 2

qualitative defects in vWF with many subtypes

VW disease type 3

severe deficiency in vWF, resulting in significant bleeding.

VWF testing would show

Plt count normal

bleeding time longer

PT normal

PTT normal to upper end

vWF:ristocetin cofactor low

vWF:Ag low

VIII low

FX def. testing

Plt count normal

BT normal

PT long

PTT long

F V def testing

Plt count normal
BT normal
PT long
PTT long

TT normal

Prothrombin deficiency testing shows

Plt count normal
BT normal
PT long
PTT long
TT normal

Prothrombin 20210

mutation associated with increased risk of thrombosis due to elevated prothrombin activity

Fibrinogen deficiency testing

plt count normal

BT normal

PT long

PTT long

TT long

factor XIII exhibits…

increased solubility due to lack of crosslinking

If you are doing a thrombin time and its abnormal and you suspect heparin what should be added to correct for the heparin

PSO4

Decreased fibrinogen is seen in….

• DIC

• primary and secondary fibrinolysis

• liver disease

• congenital disorders

increased fibrinogen is seen in….

• inflammatory disorders

• pregnancy

• women on oral contraceptives

Reptiliase is used when

thrombin time is prolonged and you suspect heparin influence.

reptiliase on a heprin sample should cause the PTT to

correct

Reptiliase on dysfibrinogenemia should cause the PTT to

prolong