Disorders of hemostasis
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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

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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


Disorders of hemostasis

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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