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Coagulation Disorders. HEMOSTASIS. BV Injury. Neural. Damage/contact. Contact. Coagulation Cascade. Blood Vessel Constriction. Platelet Aggregation. Primary hemostatic plug. Reduced Blood flow. Platelet Activation. Fibrin formation. Stable Hemostatic Plug. COAGULATION CASCADE.

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HEMOSTASIS

BV Injury

Neural

Damage/contact.

Contact

Coagulation

Cascade

Blood Vessel

Constriction

Platelet

Aggregation

Primary hemostatic plug

Reduced

Blood flow

Platelet

Activation

Fibrin formation

Stable Hemostatic Plug



Disorders of hemostasis
DISORDERS OF HEMOSTASIS

  • Disorders of Blood vessels

    • Scurvy, senile purpura, cushing’s syndrome, connective tissue disorders, HENOCH-SEHONLEIN Purpura

  • Disorders of Platelets

    • Thrombocytopenia-impaired production, hyper splenism, accelarated destruction

    • Functional-Thrombasthenia,Bernard –Soulier, VON WILLEBRAND’s disease

    • Platlete release defect-Aspirin, NSAID, Uremia

  • DISORDERS OF COAGULATION


Coagulation disorders1
COAGULATION DISORDERS

  • Inherited disorders

    • Hemophilia A and B

    • Other factor deficiencies[V,VII,X,II,XIII]

  • Acquired disorders

    • Liver disease

    • Vitamin K deficiency/warfarin overdose

    • DIC


Haemophili a
HAEMOPHILI A

  • Hemophilia was known as the “Royal disease” because it spread to the royal families of Europe through Britain Queen Victoria’s descendants




Clinical manifestations
Clinical manifestations

  • Hemophilia A & B indistinguishable

  • Hemarthrosis (most common)

    Acute or chronic

    • Fixed joints(target joints)

    • Mainly affect knees, elbows, ankles, shoulders, and hips

  • Soft tissue hematomas

    • Muscle atrophy

    • Shortened tendons

  • Other sites of bleeding

    • Urinary tract

    • CNS

    • Retroperitoneal bleed

  • Prolonged bleeding after surgery or dental extractions




Lab investigations
LAB INVESTIGATIONS

  • Normal bleeding time and platelets

  • Normal prothrombin time

  • Isolated APTT prolongation

  • Measurement of factor levels


Treatment of hemophilia a
TREATMENT OF HEMOPHILIA A

Recombinant factor VIII

  • One unit of F VIII increases the plasma F VIII level by 2%

  • The FVIII half-life of 8–12 h ;requires injections twice a day

  • Dose: 15-50 IU/d depending on the severity

    Cryoprecipitate

  • Contains half of the FVIII activity in 1/10th volume


Treatment
TREATMENT

Desmopressin “DDAVP”

  • Synthetic vasopressin analogue

  • Transient rise in FVIII and von Willebrand factor (vWF), through a mechanism involving release from endothelial cells

  • useful in mild haemophilia

    ANTIFIBRINOLYTIC DRUGS

  • Aminocaproic acid (EACA) or tranexamic acid


Treatment of hemophilia b
TREATMENT OF HEMOPHILIA B

  • Recombinant factor IX

    plasma t1/2 24 hours

    50-100 IU/Kg/day

  • FFP


Vitamin k
VITAMIN K

  • Source of vitamin K Green vegetables Synthesized by

    intestinal flora

  • Required for synthesis Factors II, VII, IX,X, Protein C and S

  • Causes of deficiency Malnutrition Biliary obstruction Malabsorption Antibiotic therapy




Lab investigations1
LAB INVESTIGATIONS

  • Normal platelets

  • Prolonged prothrombin time

  • Normal bleeding time

  • Prolongation of APTT


Treatment1
TREATMENT

Vitamin K

Fresh frozen plasma


Liver disease
LIVER DISEASE

  • Decreased synthesis of II, VII, IX, X, XI, and fibrinogen

  • Often complicated by

    • Gastritis, esophageal varices, DIC

  • Treatment

    • Fresh-frozen plasma infusion (immediate but temporary effect)

    • Vitamin K (usually ineffective)


Disseminated intravascular coagulation dic
Disseminated Intravascular Coagulation (DIC)

  • Clinicopathologic syndrome characterized by widespread intravascular fibrin formation in response to excessive blood protease activity that overcomes the natural anticoagulant mechanisms


Causes
CAUSES

  • Sepsis

  • Trauma

    • Head injury

    • Fat embolism

  • Malignancy

  • Obstetrical complications

    • Amniotic fluid embolism

    • Abruptio placentae

  • Vascular disorders

  • Reaction to toxin (e.g. snake venom, drugs)

  • Immunologic disorders

    • Severe allergic reaction

    • Transplant rejection

  • Drugs

  • Liver disease


Mechanism
MECHANISM

Systemic activation

of coagulation

Depletion of platelets

and coagulation factors

Intravascular

deposition of fibrin

Thrombosis of small

and midsize vessels

with organ failure

Bleeding


Clinical manifestations1
CLINICAL MANIFESTATIONS

  • Bleeding tendency in presence of widespread coagulation

    • Acute D.I.C.= dominated by a bleeding

      • seen in obstetrical complications and trauma

    • Chronic D.I.C.= presents with Thrombotic complications

      • seen in cancers


Clinical manifestations2
CLINICAL MANIFESTATIONS

  • Organ damage due to Micro thrombi

    • Kidney : Microinfarcts in the renal cortex

      • In severe cases = bilateral renal cortical necrosis

    • Adrenals : Bilateral adrenal hemorrhage

      • resembles Waterhouse - Friderichsen syndrome

    • Brain : Microinfarcts surrounded by foci of hemorrhage

    • Heart and anterior pituitary: show similar changes


Lab investigations2
LAB INVESTIGATIONS

  • Decreased platelets

  • Prolonged prothrombin time

  • Prolonged bleeding time

  • Prolongation of APTT

  • Elevated D dimer

  • Elevated fibrin degaradation products(FDP)


Treatment2
TREATMENT

  • Treatment of underlying disorder

  • Anticoagulation with heparin

  • Platelet transfusion

  • Fresh frozen plasma



HMWK

VII

XII

PK

XI

APTT

PT

IX

VIII

X

PT - 

APTT, TT, PLC - N

V

TT

II

I

  • Factor VII deficiency

  • Anticoagulant therapy

  • Early vitamin k deficiency


HMWK

VII

XII

PK

XI

APTT

PT

IX

VIII

X

APTT - 

PT, TT, PLC - N

V

TT

II

I

  • Factor deficiency

  • Inhibitors

  • Heparin therapy


HMWK

VII

XII

PK

XI

APTT

PT

IX

VIII

X

V

PT, APTT - 

TT, PLC - N

TT

II

I

  • Common Pathway Factor deficiency

  • Vitamin K deficiency-late

  • Oral anticoagulant therapy

  • Liver disease


HMWK

VII

XII

PK

XI

APTT

PT

IX

VIII

X

V

PT, APTT, TT - 

PLC - N

TT

II

I

  • Hypo / dysfibrinogenemia

  • Heparin

  • Liver disease

  • Systemic hyperfibrinolysis


HMWK

VII

XII

PK

XI

APTT

PT

IX

VIII

X

APTT, PT,TT all 

PLC - low

V

TT

II

I

  • DIC

  • - FDP (fibrin degradation product)

  • - D-dimer

  • - Fibrin monomer


Platelet, BT,PT, APTT- NORMAL

Causes

  • Factor XIII deficiency

  • Thrombasthenia (disorder of platelets)

    • congenital

    • drug induced

  • Disorders of vascular hemostasis


Factor XIII deficiency

  • Factor XIII- clot stabilizing factor

  • Diagnosed by clot solubility test (5 M urea solution)



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