Disorders of Secondary Hemostasis (Coagulation Pathway Factor Deficiencies)

Question 1

Common Pathway.
Autosomal Recessive Disorder.
Afribinogenemia (no fibrinogen) severe at birth.
Hypofibrinogenemia (levels between 20-100 mg/dL) few bleeding symptoms.
Lab:
Afibrinogenemia:
Increased PT, APTT, TCT, and decreased fibrinogen.
Hypofibrinogenemia: Normal PT, APTT, and Fibrinogen, Abnormal TCT.

Answer 1

Factor I Deficiency

*on test

Question 2

Common Pathway.
Autosomal Recessive Disorder.
Mild hemorrhaging. Vitamin K deficiency and liver disease)
Lab:
Increased PT and APTT.
Normal TCT and BT.
(The rarest bleeding disorder)

Answer 2

Factor II (Prothrombin) Deficiency

Question 3

Common Pathway.
Autosomal Recessive Disorders.
Mild to moderate bleeding and bruising.
Lab:
Increased PT and APTT.
Normal TCT

Answer 3

Factor V (proaccelerin) Deficiency

Question 4

Extrinsic Pathway.
Autosomal Recessive.
Mild to moderate bleeding.
Lab:
Increased PT (*only coag factor deficiency in which the PT alone is prolonged)
Normal APTT and TT.

Answer 4

Factor VII (proconvertin) Deficiency

*on test

Question 5

Common Pathway.
Autosomal Recessive Disorder.
Mild to severe bleeding.
Lab:
Increased PT and APTT
Normal TCT
(May want to exclude Vitamin K. deficiency before diagnosis).

Answer 5

Factor X (Stuart) Deficiency

Question 6

Intrinsic Pathway.
Autosomal Recessive Disorder. 
AKA Hemophilia C.
mild bleeding after trauma.
(After vWF disease, this is the most common bleeding disorder in females, jews)
Lab:
Normal PT and TT.
Increased APTT.

Answer 6

Factor XI (Plasma thromboplastin antecedent) Deficiency

Aka Hemophilia C

Question 7

Intrinsic Pathway.
Autosomal Recessive Disorder.
*Asymptomatic
*NO bleeding clinically.
Lab:
Normal PT, TT
Increased APTT

Answer 7

Factor XII (Hageman) Deficiency

*on test definitely

Question 8

Common Pathway.
Autosomal Recessive Disorder.
Umbilical cord bleeding and delayed healing.
Lab: Normal PT, APTT, and TCT.
Diagnose by placing fibrin clot in 1% monochloroacetic acid or urea, positive if it dissolves in .5ug/dL or less. Clot is insoluble at levels as low as 1-2 ug/dL

Answer 8

Factor XIII (fibrin-stabilizing) Deficiency

*def on test

Question 9

Intrinsic Pathway.
Autosomal Recessive Disorder.
Asymptomatic (no bleeding even after severe trauma or surgery).
Lab:
Normal PT and TT.
Increased APTT.

Answer 9

Prekallikrein (Fletcher) Deficiency

Question 10

Intrinsic Pathway.
Autosomal Recessive Disorder.
Asymptomatic.
Lab:
Normal PT and TT
Increased APTT

Answer 10

HMWK (Fitzgerald) Deficiency

Question 11

Intrinsic Pathway.
Sex-linked Disorder.
Aka Hemophilia A, Classic Hemophilia
Second most common deficiency, males, royal families in Europe.
Bleeding in joints, mild bleeding, easy bruising that can be severe.
Lab:
Normal PT
Increased APTT
Musculoskeletal lesions, neurological deficiencies from intracranial hemorrhage.

Answer 11

Factor VIII (Antihemophilic) Deficiency

Question 12

Intrinsic Pathway.
Sex-Linked Disorder.
Aka hemophilia B, christmas disease.
Bruising, bleeding in joints, mild bleeding.
Third most common disorder.
Lab:
Normal PT
Increased APTT

Answer 12

Factor IX (plasma thromboplastin component PTC) Deficiency

Question 13

Primary or Secondary Hemostasis Problem.
Autosomal Dominant Disorder.
Most common inherited bleeding disorder.
Primary Hemostasis: plts are intrinsically normal but exhibit abnormal adhesion because of its absence.
Secondary Hemostasis: Absence or dysfunction of this results in decreases in Factor VIII and abnormal secondary hemostasis.
Mild to severe hemorrhaging. Lab: 
Abnormal BT, APTT
Normal plt count and PT.
Specific Lab Tests (Have to know):
1. Measurments of plasma vWF antigen.
2. Factor VIII activity.
3. Assays of vWF plasma activity
**ALL DECREASED!!!
DECREASED for ABSENT with RISTOCETIN!!!

Answer 13

von Willebrand Disease

Question 14

Acquired Disorder.
Diverse group of disease states but most common diseases are infections(septicemia) and complications of pregnancy.
Lab:
Abnormal PT, APTT, TT, plt count, plt function.
FDPs +
D-dimer +
Fibrinogen decreased
Plasminogen decreased
*shistocytes

Answer 14

DIC

Question 15

PT or APTT after mixing study:
Correction
2 hour incubation PT or APTT:
Correction

Answer 15

Factor Deficiency

Question 16

PT or APTT after mixing study:
NO Correction
2 hour incubation PT or APTT:
NO Correction

Answer 16

Lupus anticoagulant

Question 17

PT or APTT after mixing study:
Correction
2 hour incubation PT or APTT:
NO Correction

Answer 17

Specific Inhibitor (VIII)