TABLE 36-1
Components of the Hemostatic System
Common
Factor
Name
1
Number
Associated Disease(s)
Hemorrhagic/
2
Thrombotic Risk
Disease
Prevalence
Concentration
Tissue
in Plasma
3
Source/Half-life (hr)
mg/L
juM
PDB
Codes for
Proteins
4
SwissProt
Designations
4
Plasma Coagulation Factors - Procoagulant Subsystem Components
Fibrinogen
I
Afibrinogenemia, dysfibrinogenemias,
increased thrombotic risk with elevated
fibrinogen concentration
Uncommon
-2,500
-7.5
Liver
72-96
3FIB,1FIC
1FID, 1FZA,
1FZD
FIBA_HUMAN
FIBB_HUMAN
FIBGJHUMAN
Prothrombin
II
5
Dysprothrombinemias, defective
thrombin function, increased risk with
some polymorphisms
Extremely rare
100
1.5
Liver
-72
2PF1, 2PF2,
2HPP, 2HPQ,
1AOH, 1PPB
THRB_HUMAN
Thromboplastin
III
6
Not applicable,
(Exogenous material containing both
tissue factor and phospholipids)
NA
NA
Homogenate of
brain or lung in
physiological
NaCl (± CaCl2)
NA
NA
Tissue Factor (TF)
III
6
—
—
Membrane protein
1BOY, 1DAN,
2HFT
TF_HUMAN
Ca2+
IV
—
—
3.3 mM (total)
1.5 mM (free)
—
Not
applicable
NA
Proaccelerin
Accelerator
Globulin (Ac-G)
V
Factor V deficiency, (parahemophilia)
Increased risk for thrombosis (Factor V
Leiden)
Extremely rare
7-10
0.025
Liver,
megakaryocytes,
endothelial cells
11-15
FA5JHUMAN
7
Abandoned
VI
F-VI is the activated form of F-V
—
—
—
NA
Proconvertin,
Plasma
thromboplastin
VII
Factor VII deficiency, increased risk of
thrombosis with elevated F-VII
concentration
Rare; 2 per
1
,
0 0 0 , 0 0 0
0.5
0.01
Liver
4-5
1DAN
FA7JHUMAN
component, (PTC)
Antihemophilic
factor (AHF),
VIII
Hemophilia A, “classical hemophilia”
1 0 0 - 2 0 0
per
1
,
0 0 0 , 0 0 0
0
.
0 0 1
-
0 . 0 0 2
Liver
8-12
1CFG, 1FAC
FA
8
JHUMAN
antihemophilic
globulin (AHG)
Christmas factor,
antihemophilic
factor В
IX
Hemophilia B, Christmas disease
20-30 per
1
,
0 0 0 , 0 0 0
5
0.1
Liver
12-24
1CFH, 1CFI,
1EDM, 1IXA,
1PFX
FA9_HUMAN
(continued)
