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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)

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disorders of hemostasis

DISORDERS OF HEMOSTASIS

Dept.of Hematology ZhongShan Hospital,FuDan University

Prof. JlAN-MIN XU

general considerations
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 considerations1
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 considerations2
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
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 coagulation1
Normal hemostasis and coagulation

2. platelet:

(1)adhesion:

to subendothelial connective tissue

by GPⅠb、vWF

(2)aggregation :

formation of platelet thrombus

by GPⅡb/Ⅲa

(3)release:

phosphatide-Arachidonic Acid -TXA2

further platelet activation and adhesion

violent vessel spasm

normal hemostasis and coagulation2
Normal hemostasis and coagulation
  • coagulation:

(1) Rearrangement of platelet membrane

phosphatidate-PF3

(2) Tissue injury- TF release

(3) Subendothelial connective tissue-Ⅻ-Ⅻa

triggering intrinsic and extrinsic pathway-white thrombus-red thrombus

normal hemostasis and coagulation3
Normal hemostasis and coagulation
  • coagulation:
    • nature: a cascade of enzymes activation

not active before activated

    • component:
      • 12 coagulation factor:

11 in plasma (tissue factor)

protein ( exclude Ca++ )

code with Ⅰ、Ⅱ、Ⅲ、Ⅳ etc.

      • PK
      • HMWK
normal hemostasis and coagulation4
Normal hemostasis and coagulation

3. course:

(1)thromboplatin

intrinsic: Ⅻ-Ⅻa——Ⅺ-Ⅺa-Ⅸ-

Ⅸa+Ⅷ+Ca+PF3

extrinsic: III-VII+Ca

Ⅹ-Ⅹa+Ⅴ+Ca+PF3

(2)thrombin:prothrombinthromboplatin+Cathrombin

slide10

Xa

Va

Ca2+

PL

Xa因子的核心作用

内源性系统

外源性系统

XII

XIIa

细胞的促凝血酶原激酶

XIa

XI

VII

VIIa

IX

IXa

Ca2+

VIIIa

Ca2+

PL

X

Xa

凝血酶

IIa

II

XIIIa

纤维蛋白原

溶解性纤维蛋白

纤维蛋白(凝块)

Boneu et al. Sang Thrombose Vaisseaux. 1998;10:291-313.

normal hemostasis and coagulation5
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 coagulation6
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

Ⅱa. Ⅻa. Ⅺa. Ⅸa. DK

normal hemostasis and coagulation7
Normal hemostasis and coagulation

(3)protein C system:

PCTMAPC+PS

sterilizeVa. VIIIa.

Inhibit PAI

physiology and pathology
Physiology and Pathology

APCI Ⅹa,trypsinize, TM+thrombin

activator sterilize Ⅴa,Ⅷa

PC APC release plasminogen

Ca++ →fibrolysis

Ca++

PSmodulate the activation of c’

acceleration inhibition

APC:Activated Protein C

normal hemostasis and coagulation8
Normal hemostasis and coagulation
  • fibrinolysis:
  • plasminogenplasminogen activator plasmin

(PAI)(a2-AP)

2. fibrinolysis:

fibrinogen→fragmentX →fragmentY →fragmentE

↘ ↘ ↘

(FDP)polypeptideABC fragmentD fragmentD

abnormality
Abnormality

1. Vessel wall:

heredity:Telangiectasis

aquired:deficiency of Vit C、 Vit P

hypersensitivity:anaphylactoid purpura

else:

abnormality1
Abnormality

2. Platelet:

(1)thrombocytopenia:

production:AA, leukemia, infection, drug

destruction :ITP

exhaust:TTP、HUS、DIC

abnormality2
Abnormality

(2)thrombocythemia :

primary:Primary hemorrhagic

thrombocythemia

secondary:CML, splenectomy , infection

trauma

(3)function deficiency:

heredity :thrombasthenia

secondary:drug, renal failure

liver disease

abnormality3
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
Diagnosis
  • History and physical exam(PE):

1.history:cause, sex、heredity background

course

2.PE:petechial

bleeding (organ, muscle, joint cavity)

diagnosis1
Diagnosis
  • Lab examination:

1.filter:

vessel, platelet: BT、plt、CRT

coagulation:CT、APTT、PT、TT

diagnosis2
Diagnosis

2.particular:

(1)platelet:

morphology:bulk、scatter、gathering

adhesion、aggregation:

inducer:Adr、ADP、collagen、ristocetin

↓ thrombasthenia

↑thrombus disease

diagnosis3
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
Prevention and Therapy

1. Prevention:

drug:aspirin, persantin, indomethacin

disease: liver disease ,renal failure

heredity:prevent traumer 、operation

prevention and therapy1
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

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