Haemostatic Failure

Presenter: Chipalavela Rossana

Facilitator: Prof Kitonyi

 Learning objectives

Understand the key states that lead to

haemostatic failure

Demonstrate ability to diagnose haemostatic

failure

Acquire key concepts of management of cases of

haemostatic failure
Normal hemostasis
 Haemostatic Failure
● Bleeding Thrombosis
– Inherited ● Risk factors
Coagulation defects
Prophylaxis
●
●
● Platelet deficiencies
● Vascular abnormalities
● Thrombophilias
– Acquired
● Liver disease
● DIC
● Vitamin k deficiency
● anticoagulantes
Inherited coagulation defects
Inherited coagulation defects
Von Willebrand disease
 TREATMENT
● Haemophilia A ● VW disease
– Factor VIII – Desmopressin
replacement – Recombinat VwF
– Plasma – VwF/FVIII
cryoprecipitates concentrates.
● Haemophilia B
– FFP
– Plasma enriched with
factor IX
 Haemophilia and surgery
● Perform in center that can monitor response to
replacement therapty

● Dosing interval for HA is 12 hrs and HB is

“24hrs

● Aim is normal factors (100%) during surgery

and subnormal (50) post op until wound healing
 Acquired coagulation defects
Liver disease
● Defective synthesis of coagulations factors
– Vit K dependent factors and others

● Thrombocytopenia
– Due to portal hypertension and splenomegaly

● Intravascular coagulation
– Reduced synthesis of ant III and Protein C
 Acquired coagulation defects
Liver disease- lab
Parameter Result
● PT and INR prolonged
● Platelet number and function Low
● Bleeding time Prolonged
● Fibrinogen and Low
individual factor levels
 Acquired coagulation defects
Liver disease- Treatment
● Coagulation defects
– FFP or factor concentrates
● Thrombocytopenia
– Platelet concentrates
● Increased fibrinolysis
– Antifibrinolytics
– FFP
 DIC
● Systemic process producing both thrombosis
and haemorrhage
● Its initiated by a number of defined disorders
– Exposure of blood to procoagulants such as tissue
factor and cancer procoagulant
– Formation of fibrin within the circulation
– Fibrinolysis
– Depletion of clotting factors
– End- organ damage
Diagnosis of DIC
 DIC Management
● Correct underlying disease and initiating factor
● Replacement therapy
– FFP
– Platelet transfusion
– Inhibit clotting process
● Tranexamic acid
● Heparin
 Vitamin k deficiency
Fat soluble vitamin

Co-factor in formation of 6 prothrombin complex

proteins ( II, VII, IX, X, protein c and S)

Obteined from green vegetables and absorbed in

the small intestines and stored in the liver

Endogenous synthesis by the bacteria in the

colon
 Platelet Disorders
● Failure of production
– Megakaryocyte depression ( drugs, chemicals,
virus)
– BM failure ( cytotoxic drugs, neoplams, HIV)
● Abnormal distribution
– splenomegaly
● Increased consumption
– Immune
– DIC
 Platelet disorders
● Dilutional loss
– Massive transfusion

● Platelet dysfunction
– Congenital ( Glanzmann´s syndrome, Bernard-
Soulier)
– Acquired ( Drugs, aspirin)
Platelets disorders
 Diagnosis of platelet disorders
Blood count film
● Low platelet ● Normal Platelet count
– Bone marrow – Bleeding time
examination – Platelet aggregation
– Platelet antibodies studies with ADP,
– Screening tests for adrenaline, collagen
DIC and ristocetin
– Other special platelet
tests, e.g. adhesion
studies, nucleotide
pool measurement
 Vascular abnormalities
● Inherited ● Acquired
– Osler weber Rendu – Henoch Schnonlein
Disease purpura
– Inherited disorders of – Haemolitic uraemic
connective tissue syndrome
matrix – Drug reaction
– infections
Thrombosis
 Thrombosis
screening tests
● Blood count
● PT and APTT ( shortened)
● D- dimers
 Thrombosis
treatment
● Antiplatelets ● Anticoagulants
– Aspirin – Heparin
– Clopidogrel ● UH
– GPIIb/IIIa inhibitors
● LMWH
– Coumarins
● Thrombolytics ● Warfarin
– New drugs
– streptokinase
● Fxa inhibitors
● Direct thrombin
inhibitors
 References
● Robbins basic pathology

● Up to date, 2017

● Harsh- Mohan- text book of Pathology.