Disorders of Coagulation

Disorders of coagulation
1. Normal haemostasis
Haemostasis is the process whereby haemorrhage
following vascular injury is arrested. It depends on
closely linked interaction between:
the vessel wall;
platelets;
coagulation factors.
The fibrinolytic system and inhibitors of coagulation
ensure coagulation is limited to the site of injury.
Haemorragic diatheses
Vasopathies (vessel wall dysfunction)
Thrombocytopenias/-pathias
Coagulopathies (inherited, aquired)
Mixed type (vWD, DIC)
Coagulation factors
are proenzymes (serine proteases) and procofactors
which are activated sequentially.
The cascade has been divided on the basis of
laboratory tests into intrinsic, extrinsic and common
pathways
Laboratory tests of coagulation.
Screening test (normal Abnormalities indicated Most common cause of
range) (prolonged abnormal) disorder
Prothrombin time (PT) (10– Extrinsic and common Liver disease, Warfarin
14s) coagulation pathways therapy, DIC
Deficiency/inhibition of
factor VII, factors X,
V, II and fibrinogen
Activated partial Intrinsic and common Heparin therapy,

thromblastin time (APTT or coagulation pathways haemophilia A and B, DIC
PTTK) (30–40s) Deficiency/inhibition of one
or more of factors XII, IX,
VIII, X, V, II and fibrinogen
Thrombin time (14–16s) Deficiency or abnormality of DIC, heparin therapy,

fibrinogen; fibrinolytic therapy
inhibition of thrombin by
heparin or FDPs
Fibrin degradation products Accelerated destruction of DIC

(<10mg/mL) fibrinogen
Platelet aggregation tests Abnormal platelet function Drugs (e.g. aspirin), uraemia,
von Willebrand’s disease
Disorders of coagulation I:
inherited
Factor VIII deficiency
(haemophilia A)
Clinical features
Range from severe spontaneous bleeding, especially
into joints (haemarthroses) and muscles, to mild
symptoms.
Onset in early childhood.
Increased risk of postoperative or post-traumatic
haemorrhage.
Chronic debilitating joint disease caused by repeated
bleeds.
Severity
Mild (>5% activity of the f.VIII)
Moderate (2-5%)
Severe (<2%)
All the lab tests normalize at the activity level of >10%.

Patients with mild form don’t have spontaneous
bleeding.
Laboratory features
Prolonged activated partial thromboplastin time
(APTT), normal prothrombin time (PT), normal
bleeding time,
Plasma factor VIII reduced
Carriers have factor VIII approximately 50% of normal.

DNA analysis is helpful in carrier detection and
counselling.
Von Willebrand factor level is normal.
Treatment
Plasma exchange, cryoprecipitate, native plasma
concentrate,
Infusions of factor VIII concentrate to elevate the patient’s
level to 20–50% of normal for severe bleeding.
Level is raised to and maintained at 80–100% for elective surgery.
Desmopressin, an analogue of vasopressin, leads to a modest
rise in endogenous factor VIII which is useful in mild cases.
Avoid aspirin, other antiplatelet drugs and intramuscular
injections.
Patients should be registered with a recognized haemophilia
centre and should carry a card with details of their condition.
Complications of treatment
HIV and hepatitis C from impure preparations,
subsequent AIDS, hepatitis and cirrhosis.
Neutralizing antibodies to factor VIII in 15% of severe
patients may require immunosuppressive therapy,
treatment with porcine factor VIII (during a week -
max), Feiba, Novoseven
Von Willebrand’s disease
Is usually autosomal dominant, results from
mutations in the von Willebrand factor (vWF) gene.
Von Willebrand factor is a large multimeric protein
produced by endothelial cells, which carries factor VIII
in plasma and mediates platelet adhesion to
endothelium.
The disease is more frequent than haemophilia A;
males and females are affected equally.
Clinical features
Bleeding, typically from mucous membranes (mouth,
epistaxes, menorrhagia).
Excess blood loss following trauma or surgery.
Haemarthroses and muscle bleeding are rare.
Diagnosis
APTT is prolonged, PT normal.
Factor VIII and vWF levels are reduced.
Bleeding time is prolonged.
Defective platelet function, reduced aggregation with
ristocetin.
Mild thrombocytopenia may occur.
Treatment
Intermediate purity factor VIII concentrate (contains
both vWF and factor VIII) for bleeding.
Desmopressin is helpful for mild bleeding.
Fibrinolytic inhibitors (e.g. tranexamic acid) are
helpful.
Disorders of coagulation II: acquired
Vitamin K deficiency (liver disease , mechanical
jaundice)
Disseminated intravascular coagulation
Drugs (warfarin)
Acquired coagulation inhibitors
Thank you for your attention.
Questions are welcome!

Disorders of Coagulation

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Disorders of coagulation

Activated partial Intrinsic and common Heparin therapy,

Thrombin time (14–16s) Deficiency or abnormality of DIC, heparin therapy,

Fibrin degradation products Accelerated destruction of DIC

All the lab tests normalize at the activity level of >10%.

Carriers have factor VIII approximately 50% of normal.

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