Transcript
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Haemostasis&Bleeding disorders
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Haemostasis
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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
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Classification:
Disorders of Blood vessels
Disorders of Platelets
Disorders of Coagulation
Other disorders
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Haemostasis
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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
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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
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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
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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
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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
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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
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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
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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
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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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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
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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
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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
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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
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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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Platelet Aggregation Curves
Haemostasis
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Combined Primary and
Secondary Hemostatic
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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Combined Primary and
Secondary Hemostatic
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
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Summary Hemostatic
Disorders
BT Plt PT PTT
Vascular Dis - - - -
PLT Disorder - - - -
Factor 8/9*Congenital - - -
Haemostasis
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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
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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
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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
Lab TestsCBC-PltBT,(CT)PT, PTT,TTSpecial tests
Bl. Disorders:Cong/AcquredBV, Coag, PLT
Haemostasis
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Nail bed - Hematoma
Red
Blue/Gr
Brown
Haemostasis
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Contusion - Hematoma
Platelet
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Platelet
Coagulation
Petechiae, Purpura Hematoma, Joint bl.
Haemostasis
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Haemostasis
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Petechiae & Echymoses -Plt
Haemostasis
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Haemostasis
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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
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