TABLE 36-1
Components of the Hemostatic System
(continued)
Common
Factor
Name1
Number 2
Associated Disease(s)
Hemorrhagic/
Thrombotic Risk
Disease
Prevalence
Concentration
in Plasma3
mg/L
jitM
Tissue
Source/Half-life (hr)
PDB
Codes for
Proteins 4
SwissProt
Designations 4
Plasma Coagulation Factors - Procoagulant Subsystem Components
(continued)
Stuart-Prower
factor (Autopro-
thrombin III)
X
Factor X deficiency
Very rare
8
0.15
Liver
24-50
1APO, 1CCF,
1FAX, 1HCG,
1KIG, 1WHE,
1WHF
FA10JHUMAN
Plasma
thromboplastin
antecedent (PTA)
XI
Factor XI deficiency
Rare, primarily
in Ashkenazi
Jews
4
0.025
Liver
40-80
FA11_HUMAN
Hageman factor
XII
None observed
NA
30
0.4
Liver
50-70
FA12_HUMAN
Fibrin stabilizing
factor, Plasma
transglutaminase
Laki-Lorand
factor,
fibrinoligase
XIII
Spontaneous abortion, poor
wound healing, excessive scar
tissue formation
Very rare
30
1 1
(a subunit)
2 1
(b subunit)
Liver 100-200
1FIE, 1F13,
1GGT
F13A_HUMAN
F13B_HUMAN
Prekallikrein
Fletcher factor
None
None observed
—
50
0 . 6
Liver
35
1HIA, 2KAI
KAL_HUMAN
High Mol Wt
Kininogen
Fitzgerald factor,
Flaujac factor,
Williams factor
None
None observed
70
0.7
Liver
150
KNHHUMAN
von Willebrand
factor
None
von Willebrand’s disease,
many forms
1-125 per
1
,
0 0 0 , 0 0 0
5-10
8
Endothelial cells,
megakaryocytes/
~ 1 2
(multimer)
~ 2
(propeptide)
1A03, 1ATZ,
1AUQ, 10AK
VWF_HUMAN
(continued)
