COAGULOPATHY

AN ENIGMA IN SURGICAL
PRACTICE
DR.GAUTAM MUKHOPADHYAY
DISCUSSION INTO TWO BROAD HEADINGS

DISORDER IN HEMOSTASIS FRANK COAGULOPATHY

Vessel wall disorder Disorder in platelet function
Other associated factors: Disorder in clotting mechanism
*Von Willebrand factor Other associated factors
 HEMOSTASIS
Subendothelial injury + { V.W.F. + collagen}

Platelet activation

Activated platelet

Site for coagulation factor activation

Activated fibrin formation

Hemostasis
 HEMOSTASIS
Complex process
BALANCE BETWEEN
vasodilator and vasoconstrictor properties of injured vessel wall
vs
antithrombotic and prothrombotic component proteins in the plasma

Platelet function and vessel wall endothelium state plays significant

role
 PLATELET FORMATION
ORIGIN: Liver sinuses from
megakaryocyte mature platelet
(plasma thrombopoitin)

Inflammatory modulator: IL-6 (function:thrombocytosis)

 PLATELET AGGREGATION
Injury or insult brings about activation

Cytoplasmic granules having adhesive protein growth factor and

procoagulants platelet aggregation

Clot formation (stabilized by fibrin mesh) occlusive thrombin

 VESSEL WALL ENDOTHELIUM
Normally inactivate platelet

Help in fibrinolysis

Coagulation inhibition in normal endothelial lining

Nitric oxide: endothelial (vasodilator effect)
coagulation (inhibitory effect)
Injured endothelium: secrete endothelin (platelet activation
hemostasis casade completed)
 DISORDERS OF HEMOSTASIS
PLATELET DISORDERS
Thrombocytopenia
a. inherited b. acquired
Pseudothrombocytopenia
sequestration of platelets in spleen
platelet destruction
Drug induced thrombocytopenia
Infection induced thrombocytopenia
Immune thrombocytopenic purpura
thrombocytosis
QUALITATIVE DISORDERS OF PLATELET FUNCTION

Platelet storage pool disorder

Autosomal recessive disorder Bernard-Soulier Syndrome
Glanzmans thromboasthenia

Aquired platelet dysfunction High dose penicillin

Anti-platelet therapy
VON WILLEBRAND DISEASE (VWF)
Most common inherited bleeding disorder
3 major types and 4 subtypes in type 2 (type 1-most common)
VWF has 2 important components
MAJOR adhesive molecule function(platelet like)
Factor VIII half life related
 VWF CLASSIFICATION
TYPE 1: a.VWF protein
b.VWF function deranged
c.concomitant factor VII activity
TYPE 2: A/B/M/N
A,B,M- Platelet and collagen binding activity
N- Mutation of VWF
TREATMENT: Desmopressin(DDAVP) i.v. / intranasal spray
TYPE 3: VWF replacement required
 DISORDER IN VESSEL WALL
INHERITED DISORDER (vasculitis/CTD/vessel wall abnormality)

METABOLIC & INFLAMMATORY DISORDER (AFI/immune complex with

virus/polyclonal gammopathy:Multiple Myeloma)

VITAMIN C DEFICIENCY STATE (disorder in collagen

synthesis/hydroxyprolene)

CUSHING SYNDROME (long-term glucocorticoid therapy)

HENOCH SCHONLEIN / ANAPHYLACTOID PURPURA (compliment

component in capillaries pathology)
 COAGULATION DISORDERS
WELL-KNOWN FACT FOR CENTURIES
POST TRAUMA OR SPONTANEOUS BLEEDING EPISODES INTO
JOINTS/MUSCLES/CLOSED SPACES
MOST COMMON FACTORS INVOLVED:
a. deficiency of Factor VIII (classical hemophilia A) Sex-linked
b. deficiency of Factor IX (hemophilia B)
c. deficiency of Factor XI (hemophilia C)
OTHER FACTORS : Factor II (prothrombin)
Factor V (proaccelarin/labile factor)
Factor VII (proconvertin/stable factor)
Factor X (Stuart Prower Factor)
Factor XIII(Laki Lorand Factor/fibrin stabilizing factor)

Factor I (fibrinogen)
Factor III (thromboplastin)
Factor IV (calcium)
Factor XII (Hageman Factor/glass factor)
HMW-K (high molecular weight kininogen)
Pre-Ka (prekallikrein)
Ka (kallikrein)
PL (platelet phospholipid)
COMBINED
COAGULATION
PATHWAY
 COAGULOPATHY IN SURGICAL AND
OBSTETRICAL PRACTICES
RETAINED BLOOD CLOTS

CONSUMPTION OF COAGULATION FACTORS (mainly Fibrinogen)

DISSEMINATED INTRAVASCULAR COAGULATION/

ACCIDENTAL HEMORRHAGE IN OBSTRETICAL CASES
 WORK-UP/MANAGEMENT
SCREENING FOR CLOTTING FACTOR ACTIVITY
Congenital:
ISOLATED ABNORMAL PT (factor VII def)
aPTT (hemophilia A/B)
PT and aPTT (def of factors I,II,V,X)
Acquired: (more common)
VITAMIN K DEFICIENCY
DIC
 DIC
CONSUMPTION COAGULOPATHY
MAY ALSO BE AS A RESULT OF PERTURBATION OF BOTH PRIMARY
AND SECONDARY FACTORS LIKE
1.Platelet and vessel wall interaction
2.Autoantibodies to plasma protein inhibitors
(rarer than classical hemophilia A/B)
MANAGEMENT: 1.ANTICOAGULANTS
(heparin preparations/epsilon amino-caprioic acid)
2. FRESH FROZEN PLASMA
 HEMOPHILIA

TRANSFUSION THERAPY NON-TRANSFUSION THERAPY

FFP DDAVP (synthetic vasopressin
CRYOPRECIPITATE OF FACTOR analogue)
VIII PROTEIN Works by increasing factor VIII and
VWF
Factor IX is not affected

COMPLICATIONS: INHIBITOR FORMATION FOR ALL THE FACTORS

 TAKE HOME MESSAGE
IN EMERGENCY SITUATION, IT IS IMPORTANT FOR US SURGEONS TO
CONTROL THE HEMORRHAGE AT FIRST F/B THOROUGH WORK-UP
FOR BLEEDING AND CLOTTING DISORDERS

JUDICIOUS MANAGEMENT AND STRONG ATTITUDE WILL HELP IN

RELIEVING THE AGONY AS WELL AS COMPLAINTS OF THE PATIENTS
AND THEIR FAMILY MEMBERS
THANK YOU