Coagulation disorders
Sponsored Links
This presentation is the property of its rightful owner.
1 / 37

Coagulation Disorders PowerPoint PPT Presentation


  • 85 Views
  • Uploaded on
  • Presentation posted in: General

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.

Download Presentation

Coagulation Disorders

An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -

Presentation Transcript


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


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 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

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


HAEMOPHILIA


INHERITANCE


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


HEMARTHOSES


Bleeding and clotting disorders


LAB INVESTIGATIONS

  • Normal bleeding time and platelets

  • Normal prothrombin time

  • Isolated APTT prolongation

  • Measurement of factor levels


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

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

  • Recombinant factor IX

    plasma t1/2 24 hours

    50-100 IU/Kg/day

  • FFP


VITAMIN K

  • Source of vitamin K Green vegetablesSynthesized by

    intestinal flora

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

  • Causes of deficiencyMalnutritionBiliary obstructionMalabsorptionAntibiotic therapy


SOURCES OF VITAMIN K


VITAMIN K CYCLE


LAB INVESTIGATIONS

  • Normal platelets

  • Prolonged prothrombin time

  • Normal bleeding time

  • Prolongation of APTT


TREATMENT

Vitamin K

Fresh frozen plasma


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)

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


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

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 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 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 INVESTIGATIONS

  • Decreased platelets

  • Prolonged prothrombin time

  • Prolonged bleeding time

  • Prolongation of APTT

  • Elevated D dimer

  • Elevated fibrin degaradation products(FDP)


TREATMENT

  • Treatment of underlying disorder

  • Anticoagulation with heparin

  • Platelet transfusion

  • Fresh frozen plasma


COAGULATION CASCADE AND LAB INTERPRETATION


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)


THANK YOU


  • Login