Chapter 110. Coagulation Disorders (Part 1) pot
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Chapter 110. Coagulation Disorders
(Part 1)
Harrison's Internal Medicine > Chapter 110. Coagulation Disorders
Coagulation Disorders: Introduction
Deficiencies of coagulation factors have been recognized for centuries.
Patients with genetic deficiencies of plasma coagulation factors exhibit lifelong
recurrent bleeding episodes into joints, muscles, and closed spaces, either
spontaneously or following an injury. The most common inherited factor
deficiencies are the hemophilias, X-linked diseases caused by deficiency of Factor
(F) VIII (hemophilia A) or Factor IX (FIX, hemophilia B). Rare congenital
bleeding disorders due to deficiencies of other factors, including FII
(prothrombin), FV, FVII, FX, FXI, FXIII, and fibrinogen are usually inherited in
an autosomal recessive manner (Table 110-1). Advances in characterization of the
molecular bases of clotting factor deficiencies have contributed to a better
understanding of the disease phenotypes and may allow more targeted therapeutic
approaches through the development of small molecules, recombinant proteins, or
cell and gene-based therapies.
Table 110-
1 Genetic and Laboratory Characteristics of Inherited
Coagulation Disorders
Laborator
y Abnormality
a
Clo
tting
Factor
Deficienc
y
In
heritance
Pr
evalence
in
General
Populati
on
a
PTT T T
M
inimum
Hemosta
tic
Levels
Trea
tment
P
lasma
Half-
Life
Fib
rinogen
AR
1
in
1,000,000
+
10
0 mg/dL
Cryo
precipitate
2
–4 d
Pro AR
1
+
20 FFP/ 3
thrombin in
2,000,000
–30% PCCs –4 d
Fac
tor V
AR
1
in
1,000,000
+
/– /–
15
–20%
FFP 3
6 h
Fac
tor VII
AR
1
in
500,000
–
15
–20%
FFP/
PCCs
4
–6 h
Fac
tor VIII
X-
linked
1
in 5,000
+
30
%
FVII
I
concentrate
s
8
–12 h
Fac
tor IX
X-
linked
1
in 30,000
+
30
%
FIX
concentrate
s
1
8–24 h
Fac AR
1
+
15 FFP/ 4
tor X in
1,000,000
/– /– –20% PCCs 0–60 h
Fac
tor XI
AR
1
in
1,000,000
+
15
–20%
FFP 4
0–70 h
Fac
tor XII
AR
N
D
+
b
b
6
0 h
HK
AR
N
D
+
b
b
1
50 h
Pre
kallikrein
AR
N
D
+
b
b
3
5 h
Fac
tor XIII
AR
1
in
2,000,000
–
/–
2–
5%
Cryo
precipitate
1
1–14 d
a
Values within normal range (–) or prolonged (+).
b
No risk for bleeding, treatment is not indicated.
Abbreviations: HK, high-
molecular weight kininogen; AR, autosomal
recessive; aPTT, activated partial thromboplastin time; PT, prothrombin time; TT,
thrombin time; ND,
not determined; FFP, fresh frozen plasma; PCCs, prothrombin
complex concentrates.
