hemat haemostasis

8/13/2019 Hemat Haemostasis

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Haemostasis&Bleeding disorders


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Haemostasis

1/5/2014 Thrombocytopenia 2

Introduction: Road map..

Haemostasis capacity to minimise loss ofblood following injury to blood vessel.

Blood vessel Coagulation Platelet act.

Bleeding disorders Bv, Plt, Coag.

Laboratory tests of Haemostasis.

BT, CT, PT, aPTT, TT, FDP. Factor analysis, PLT function, PCR


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Haemostasis overview:

BV Injury

Platelet

Aggregation

Platelet

Activation

Blood Vessel

Constriction

Coagulation

Cascade

Stable Hemostatic Plug

Fibrinformation

Reduced

Blood flow

Contact/

Tissue

Factor

Primary hemostatic plug

Neural


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Haemostasis


Classification:

Disorders of Blood vessels

Disorders of Platelets

Disorders of Coagulation

Other disorders


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Haemostasis


HEMOSTASIS

Primary Hemostasis Blood vessel contraction

Platelet Plug Formation

Secondary HemostasisActivation of Clotting Cascade

Deposition & Stabilization of Fibrin

Tertiary Hemostasis Dissolution of Fibrin Clot

Dependent on Plasminogen Activation


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Haemostasis:

Vasoconstriction N

Platelet activation

Haemostatic plug

Coagulation

Stable clot formation

Clot dissolution


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Coagulation:

Contact activation-Intrinsic system

Tissue factor activation

Extrinsic Common path-

amplification

Fibrin formation Fibrin lysis.


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Coagulation Cascade:

Intrinsic Pathway (Contact)(12,11,9,8)

Extrinsic PathTissue - (7)

FibrinogenFibrin

Common

Path

(5,2)

(PT)(aPTT)

(TT)

(F & FDP)

(Factor 10)

(Thrombin)


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Haemostasis


Disorders of Hemostasis

Vascular disorders

Scurvy, easy bruising,

Platelet disorders

Low Number or abnormal function

Coagulation disorders

Factor deficiency.

Mixed/Consumption: DIC


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Haemostasis


Bleeding: Clinical Features

1. Local - Vs - General, spontaneous . .2. Hematoma & Joint bleed - Coagulation3. Skin/Mucosal Petechiae & Purpura PLT

4. wound / surgical bleeding

Immediate - (PLT) Delayed - (Coagulation)


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Haemostasis


Coagulation disorders:

Deficiencies of Clotting factors

Onset - delayed after trauma

Deep bleeding Into joints - Hemarthroses

Into deep tissuesHematoma

large skin bleedEcchymoses


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Haemostasis


Coagulation Disorders

Laboratory findings:

Normal bleeding time & Plateletcount

Prolonged prothrombin time (PT)

deficiencies of II, V, VII, X

Prolonged time (aPTT) all factors except VII, XIII

Mixing studies - normal plasma

corrects PT or aPTT


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Haemostasis


Factor VIII Deficiency

Classic hemophilia (hemophilia A)

X-linked disorder (affects 1 males)

Most common - severe bleeding

Spontaneous hematomas

Abnormal aPTTIntrinsic path.

Diagnosis - factor VIII assay

Treatment - factor VIII concentrate

Cryoprecipitate (less desirable)


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Haemostasis


Factor IX Deficiency

Christmas disease (Hemophilia B)

X-linked recessive disorder

Indistinguishable from classic hemophilia

(F VIII)

Requires evaluation of factor VIII and IXactivity levels to diagnose

Treatment - factor IX concentrate Cryoprecipitate if factor IX unavailable


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Haemostasis


Secondary Hemostatic

Disorders

Acquired coagulation disorder: Vitamin K deficiency

- neonates - decreased intestinal

flora and dietary intake- oral anticoagulants (coumadin)

- fat malabsorption syndromes

Required for factors II, VII, IX, X Prolonged PT and aPTT


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Haemostasis


Haemophilia

Congenital deficiency -Factor 8 (A) or 9(B)

Bleeding Haematoma, joint etc.

Gene on X chromosome.

(Carrier females, Males suffer)

Prolonged PTTbut normal PT.

FFP or Factor replacement Life long.

i


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Haemostasis


Platelet Disorders -

Features:

Mucocutaneous bleeding

Petechiae, Purpura, Ecchymosis.

spontaneous bleeding aftertrauma

CNS bleeding (severe plt)

Prolonged bleeding time (BT)

H i


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Haemostasis


Disorders of platelets

Decreased Number:Thrombocytopenia Decreased Production Decreased SurvivalImmune (ITP) Increased utilization - DIC

Defective Platelet function:AcquiredDrugsAspirin, MPS, MDS

CongenitalEg. Thrombasthenia.

H t i


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Haemostasis


Platelet dysfunction:

Inherited Disorders:

Bernard-Soulier disease

large platelets, failure of adhesionGlanzmanns thrombasthenia

normal size, failure of aggregation

Acquired Disorders: Drugs - Aspirin, Alcohol, Uremia,

H t i


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Haemostasis


Routine Investigations: Bleeding timeBV, PLT

ivy template method - 3-8min Clotting timeinaccurate 10-20min Prothrombin timeExtrinsic (2,5,10 + 7),

Acquired diseases, liver dis, warfarin therapy aPTT Intrinsic (2,5,10 + 8,9,12)

Haemophilia, Congenital. Trombin Time:Fibrinogen (common path)

DIC & Heparin therapy. CBC PltCount FDPFibrinogen Degradation Products - DIC


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H t i


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Haemostasis


BLEEDING TIME vs.

PLATELET COUNT

0

50

100

150

200

250

300

350

400

3.5 4 4.5 5 5.5 7 9 12 15 25 30

Minutes

Plateletcoun

t(x

1000)

Haemostasis


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Haemostasis


Platelet Aggregation Curves

Haemostasis


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Haemostasis


Combined Primary and


Disorders (DIC)Disseminated IntravascularCoagulation

Major pathologic processes -obstetric complications, neoplasms,

infection (sepsis), major trauma

Primary - platelet consumption( bleeding time, platelets)

Secondary - factor consumption

( PT, aPTT)

Haemostasis

C bi d P i d


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Haemostasis


Combined Primary and


DisordersSevere Liver Disease

Primary - dysfunctional platelets

and/or thrombocytopenia ( BT) Secondary - decrease in all

coagulation factors except vWF

( PT, aPTT)Vitamin K will promote synthesis

of factors II, VII, IX, X

Haemostasis


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Haemostasis


Summary Hemostatic

Disorders

BT Plt PT PTT

Vascular Dis - - - -

PLT Disorder - - - -

Factor 8/9*Congenital - - -

Haemostasis


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Haemostasis


Summary

Symptom Platelet Coagulation

Petechiae Yes No

Sites Skin &Mucosa

Deep Tissue

Time Immediate Delayed

Ecchymoses/Hematomas

Yes Yes

Haemostasis


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Haemostasis


Special Investigations:

Specific Factor Assays Platelet function studies

Aggregometry,

Adhesion studies Immuno-fluorescence

Electrophoresis Bone marrow examinationplt

Molecular BiologyFISH

Haemostasis

L b T t


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Haemostasis


Haemostasis overview:

BV Injury

Platelet

Aggregation

Platelet

Activation

Blood Vessel

Constriction

Coagulation

Cascade

Stable Hemostatic Plug

Fibrinformation

Reduced

Blood flow

Contact/

Tissue

Factor

Primary hemostatic plug

Neural

Lab TestsCBC-PltBT,(CT)PT, PTT,TTSpecial tests

Bl. Disorders:Cong/AcquredBV, Coag, PLT

Haemostasis


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Haemostasis


Nail bed - Hematoma

Red

Blue/Gr

Brown

Haemostasis


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Haemostasis


Contusion - Hematoma

Platelet


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Platelet

Coagulation

Petechiae, Purpura Hematoma, Joint bl.

Haemostasis


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Haemostasis


Petechiae & Echymoses -Plt

Haemostasis


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Haemostasis


Petechiae & Echymoses -Plt

Haemostasis


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Bleeding-Coagulation disorder

Deep bleeding

Haematoma

Joint bleeds

Haemophilia

Haemostasis


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Sub Conjuctival Haemorrhage

Low PLT

Haemostasis

D

H h i f


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Dengue Hemorrhagic fever

Plt

Haemostasis


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Petechiae in

Vasculitis

(Rocky Mountain Spotted Fever)

Haemostasis


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Henoch-Schonlein purpura

Immunedisorder

Children

Followsinfection

Petechiae withedema and

itching.

Haemostasis


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Haemostasis


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Dengue Hemorrhagic fever

hemat haemostasis

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