DISORDERS OF
HEMOSTASIS
Dept.of Hematology ZhongShan
Hospital,FuDan University
Prof,JlAN-MIN XU
General Considerations
? Disorders of hemostasis
defects in platelet number or function
problems in fibrin clot (coagulation)
? Bleeding due to platelet disorders is
typically mucosal or dermatologic
include epistaxis,gum bleeding,
menorrhagia,gastrointestinal bleeding,
purpura,and petechiae
General Considerations
? Petechiae are seen almost exclusively in
conditions of thrombocytopenia and not
platelet dysfunction,
? Bleeding due to coagulopathy may occur
as deep muscle hematomas as well as
skin bleeding,
? Spontaneous hemarthroses are seen only
in severe hemophilia,
General Considerations
? Definition,
disorder of hemostasis
spontaneous bleeding
abnormal bleeding following trauma
? Machanism,
vessel wall defect
thrombocytopenia,platelet disfunction
coagulation disorder
Normal hemostasis and coagulation
? hemostasis,
1,Vessel wall,
（ 1） vessel spasm,reflex spasm
S-HT↑ slow down blood flow
（ 2） subendothelial connective tissue,
encouraging platelet adhesion,activation,
aggregation
（ 3） releasing TF,activating FⅫ,
triggering intrinsic and extrinsic pathway
（ 4） pression of haematoma
Normal hemostasis and coagulation
2,platelet,
（ 1） adhesion,
to subendothelial connective tissue
by GPⅠ b,vWF
（ 2） aggregation,
formation of platelet thrombus
by GPⅡ b/Ⅲ a
（ 3） release,
phosphatide-Arachidonic Acid -TXA2
further platelet activation and adhesion
violent vessel spasm
Normal hemostasis and coagulation
3,coagulation,
（ 1） Rearrangement of platelet membrane
phosphatidate-PF3
（ 2） Tissue injury- TF release
（ 3） Subendothelial connective tissue-Ⅻ -Ⅻ a
triggering intrinsic and extrinsic pathway-
white thrombus-red thrombus
Normal hemostasis and coagulation
? coagulation,
1,nature,a cascade of enzymes activation
not active before activated
2,component,
1,12 coagulation factor,
11 in plasma (tissue factor)
protein ( exclude Ca++ )
code with Ⅰ, Ⅱ, Ⅲ, Ⅳ etc,
2,PK
3,HMWK
Normal hemostasis and coagulation
3,course,
（ 1） thromboplatin
intrinsic,Ⅻ -Ⅻ a—— Ⅺ -Ⅺ a-Ⅸ -
Ⅸ a+Ⅷ +Ca+PF3
extrinsic,III-VII+Ca
Ⅹ -Ⅹ a+Ⅴ +Ca+PF3
（ 2） thrombin,prothrombinthromboplatin+Cathrombin
Boneu et al,Sang Thrombose Vaisseaux,1998;10:291-313,
II
VIIa
细胞的促凝血酶
原激酶
Ca2+
Xa
XIa
内源性系统 外源性系统
X
IX
XIIIa
XI
XII XIIa
溶解性纤维蛋白 纤维蛋白原 纤维蛋白（凝块）
Xa
Va
PL
Ca2+
VII
凝血酶
IIa
IXa
VIIIa
Ca2+
PL
Xa因子的核心作用
Normal hemostasis and coagulation
（ 3） fibrin,fibrinogenthrombinfibrin
monomer-polymerXIIIapolymer
（ 4） function of thrombin,
coagulation,activateⅠ, Ⅻ,
strengthen Ⅷ, Ⅴ
platelet,aggregation,release
fibrinolysis,activate plasminogen
Normal hemostasis and coagulation
? Anticoagulant system,
1,anticoagulin,
（ 1） each coagulate factor and their complex has
its own inhibitor
（ 2） important,
AT-Ⅲ, 70% anticoagulant activity
serine proteinase inhibitor
Ⅱ a,Ⅻ a,Ⅺ a,Ⅸ a,DK
Normal hemostasis and coagulation
（ 3） protein C system,
PCTMAPC+PS
sterilize Va,VIIIa,
Inhibit PAI
Physiology and Pathology
APCI Ⅹ a,trypsinize,
TM+thrombin
activator sterilize Ⅴ a,Ⅷ a
PC APC release plasminogen
Ca++ →fibrolysis
Ca++
PS modulate the activation of c’
acceleration inhibition
APC,Activated Protein C
Normal hemostasis and coagulation
? fibrinolysis,
1,plasminogenplasminogen activator plasmin
（ PAI）（ a2-AP）
2,fibrinolysis,
fibrinogen→fragmentX →fragmentY →fragmentE
↘ ↘ ↘
（ FDP） polypeptideABC fragmentD fragmentD
Abnormality
1,Vessel wall,
heredity,Telangiectasis
aquired,deficiency of Vit C,Vit P
hypersensitivity,anaphylactoid purpura
else,
Abnormality
2,Platelet,
（ 1） thrombocytopenia,
production,AA,leukemia,infection,drug
destruction, ITP
exhaust,TTP,HUS,DIC
Abnormality
（ 2） thrombocythemia,
primary,Primary hemorrhagic
thrombocythemia
secondary,CML,splenectomy,infection
trauma
（ 3） function deficiency,
heredity, thrombasthenia
secondary,drug,renal failure
liver disease
Abnormality
3,Coagulation,
（ 1） heredity,hemophilia,willabrand
disease
（ 2） aquired,liver disease,renal failure,
VitK deficiency,DIC
（ 3） anticoagulant agents,Ⅷ, Ⅸ -Ab,drug
Diagnosis
? History and physical exam(PE),
1.history,cause,sex,heredity background
course
2.PE,petechial
bleeding (organ,muscle,joint cavity)
Diagnosis
? Lab examination,
1.filter,
vessel,platelet,BT,plt,CRT
coagulation,CT,APTT,PT,TT
Diagnosis
2.particular,
（ 1） platelet,
morphology,bulk,scatter,gathering
adhesion,aggregation,
inducer,Adr,ADP,collagen,ristocetin
↓ thrombasthenia
↑thrombus disease
Diagnosis
（ 2） Coagulation,
APTT+CT,VIII,IX,XI
PTCT,V,VII,X,II
TT,fibrinogen↓,anticoagulant agents
Coagulant factor test,quantity
activation
Prevention and Therapy
1,Prevention,
drug,aspirin,persantin,indomethacin
disease,liver disease,renal failure
heredity,prevent traumer, operation
Prevention and Therapy
2,hemostasis,
（ 1） supplyment,coagulant factor,fresh
plasma,blood,platelet
（ 2） drug,
vessel,VitC,VitP,Carbazochrome, Pred
platelet,Etamsylate, Pred
coagulation,VitK,prothrombin complex
fibrinolysis,EACA,PAMBA,Tranexamic Acid
（ 3） local,oppression,decrease movement