DISORDERS OF

HEMOSTASIS
HASSAN ALSINAN – SBEM R2

SUPERVISED BY : DR ABDULLAH ALMARSHAD

OBJECTIVES

• Normal coagulation and pathophysiology

• The fibrinolytic system
• Evaluation of bleeding patient
• Bleeding time, prothrombin time and platelet function assay
• Testing of hemostasis
NORMAL COAGULATION AND PHYSIOLOGY
R E G U L AT I N G
C L O T F O R M AT I O N
- Prevent propagation of
coagulation pathway
- Tissue factor pathway
inhibitor
- Heparin Sulfate

- Prevent Platelet aggregation

- Prostacyclin
- Nitric oxide

- Antiplatelet and antithrombotic

- AMP ( Degradation of ATP
and ADP by CD39)

- Prevent platelet plug formation

- Adenosine ( Degradation of
ATP and ADP by CD39)
HEMOSTASIS

• Dynamic process to prevent blood from escaping vessels and a response to vessel injury
• Phases
– Maintenance of vessel integrity
– Formation of platelet plug (primary hemostasis)
– Propagation of coagulation cascade (secondary hemostasis)
– Clot development
– Fibrinolysis and clot disintegration
PRIMARY HEMOSTASIS (PLATELET
PLUG)
Platelets role:
- Exposure of damaged endothelium
- Platelets display glycoproteins
- GPIb binds exposed vWF
- GIIb/IIIa allow platelets to bind each other
- Release potent vasoconstrictors
- Thromboxane A
- Platelets aggregate over exposed area of injury
SECONDARY HEMOSTASIS
(COAGULATION PATHWAY)
COAGULATION PATHWAY
PLATELET PLUG
T H E F I B R I N O LY T I C
SYSTEM

- Limits size of fibrin clot

- Endothelial cells release

Tissue plasminogen
activator
- Plasminogen from liver

- Plasmin degrades clot

- Products : Fibrin and D-

Dimer
OTHER INHIBITORS OF HEMOSTASIS

• Antithrombin
– Binds Coagulation factors XIIa, XIa, Xa, Thrombin
– Inhibits their function
– Heparin potentiates Antithrombin
• Protein C & Protein S
– Vitamin K dependent
– Produced in liver
– Inactivates Factor Va & VIIIa
• Factor V laiden deficiency, defects in antithrombin, protein C or Protein S = hypercoagulable states
HEMOSTASIS
EVALUATION OF BLEEDING PATIENT

• Stabilization : IV fluid , Red blood cells or other blood components or factors (if known)
• History and physical exam

Nature of bleeding Petechia

Purpura
Ecchymosis
Significant bleeding episodes
Site Skin
Mucosa
Muscle
GIT
GU
Joints
HISTORY
Pattern of bleeding Recent or lifelong
Frequency & severity
Spontaneous or traumatic

Challenges Dental extraction

Operative
Medications

Associated disease Uremia : platelet dysfunction

Liver disease : Coagulation factor defecits
Infection : platelet or coagulation
Malignancy : platelet or coagulation

Previous transfusion history

Family history
EXAM
Vitals
Skin Nature of bleeding
Signs liver disease
Petechial
Purpura
Distribution

Mucosa Oral or nasal . Epistaxis

Lymphadenopathy
Abdomen Liver : size & shape
Splenomegaly

Joints Signs of previous bleeding

Other sites blood loss Pelvic, rectal , urinary tract, intramuscular , deep soft
tissue
WHAT KIND OF HEMOSTATIC
DISORDER?
• Vascular disorders
• Platelet disorders
• Coagulation disorders
VASCULAR DISORDERS
PLATELET DISORDERS
COAGULATION DISORDER
 SOME HINTS TO DIFFERENTIATE
Vascular disorder Platelet disorder Coagulation disorder
Palpable purpura Women > Men Men > Women
Itch or burn with purpura If inherited = x-linked
Connective tissue or endothelial acquired petechiae, purpura, or
damage mucosal bleeding, Source : intramuscular or deep
soft tissue hematoma
Epistaxis, menorrhagia, and
gastrointestinal bleeding are Bleeding after surgery or trauma
common Can be delayed up to 72 hrs

Occur immediately after surgery Hematuria and hemarthrosis

or dental extraction
Bleeding Time normal (except von
Willibrand)
TESTING OF HEMOSTASIS
Test Clinically
CBC Assess anemia with bleeding episode
Blood smear Schistocytes or fragmented RBC ( MAHA)
Immature WBC (leukemia)
Platelet count Thrombocytopenia If less than 10,000 high risk of
(150,000 – 400,000) spontaneous bleeding
Thrombocytosis Inflammation or malignance
Polycythemia vera
Hemorrhage or thrombosis
Bleeding time (8-10 minutes) Prolonged if : Thrombocytopenia Or abnormal platelet function
Platelet function assay Test platelet adhesion & Sensitive to detect moderate to
aggregation severe vWD. Medication related
platelet dysfunction. Severe
platelet function disorders
Test Measure Clinically
Prothrombin Time (PT) & Extrinsic & common pathway Monitor warfarin
INR (II , V, VII, X, Fibrinogen) Prolonged in liver disease
Antibiotics that inhibit Vit K
factors ( Cefotaxime)

Partial thromboplastin time Intrinsic & Common pathway Prolonged : heparin or factor
(PTT) (All except VII & XIII) deficiency (<40%)
Thrombin Time Measure conversion of fibrinogen to fibrin Fibrinogen abnormalities
Direct thrombin inhibitors
( dabigatran )

Anti Xa Assay Monitor UFH or LMWH or direct Xa inhibitor ( Rivoroxaban)

Fibrinogen Final coagulation step Low = decreased production ( liver
disease) or overconsumption (DIC)
High = Acute phase reactant

D-Dimer Breakdown of cross linked fibrin High : DIC , venous thrombus, PE ,

liver disease, pregnancy

Factor level assay Percent activity of certain factors Identify deficiency

REFERENCES
THANK YOU! QUESTIONS ?