APPROACH TO COAGULATION DISORDERS

Dr.K.Pavithran, MD, DM Assistant Professor,Dept of Hematology Medical College Hospital Trivandrum-695011, India

Clinical approach
1. 2. 3. 4. Is the bleeding significant ? Local Vs Systemic ? Platelet Vs Coagulation disorder ? Inherited Vs Acquired ?

Laboratory Approach
1. 2. 3. 4. 5. Demonstration of the defect Identification of the defect(s) Assessment of severity Consequential studies eg. carrier detection Monitoring of treatment

Screening Tests
1. Platelet count & morphology 2. Bleeding Time 3. Clotting Time 4. Prothrombin Time 5. Activated Partial Thromboplastin Time 6. Thrombin Time

Collection of blood sample

1. Minimum circulatory stasis

2. Clean venous puncture

3. Proper anticoagulant 4. Proportion of blood to anticoagulant 5. Separation of plasma and storage 6. Effect of stress, pregnancy, drugs 7. Effect of PCV on the proportion of plasma to anticoagulant

Prolonged PT/APTT
Coagulation factor deficiency/inhibitor Test plus control plasma - 1:1 Repeat PT/APTT > 50% correction
Yes - Factor deficiency No - inhibitor
timed incubation
abnormally increasing specific inhibitor no change Lupus Anticoagulant

HMWK

XII PK XI APTT IX
VIII

VII

PT
X V II I

PT - APTT, TT, PLC - N

TT

* Factor VII deficiency * Anticoagulant therapy

HMWK

XII PK XI APTT IX
VIII

VII

PT
X V II I

APTT - PT, TT, PLC - N

TT

* Factor deficiency * vWD * Inhibitors * Heparin therapy

Mixing tests with APTT

APTT of test plasma + Aged plasma Adsorbed plasma
Diagnosis

No correction
Corrected

Corrected
No correction

VIII
IX

Corrected

Corrected

XI,XII

Prolonged APTT, BT
von Willebrands disease Ristocetin Induced Platelet Agglutination VIII:C vWF:Ag vWF multimeric analysis Type 1 - Partial deficiency of vWF 2A - Absence of large and interm. multimers 2B - Absence of large multimers 2M- multimers normal, pl. function 2N - affinity for FVIII 3 - severe deficiency of vWF

HMWK

XII PK XI APTT IX
VIII

VII

PT
X

PT, APTT - TT, PLC - N

V II I TT

* * * *

Common Pathway Factor deficiency Vitamin K deficiency Oral anticoagulant therapy Liver disease

Mixing tests with PT

PT of test plasma + Aged plasma adsorbed plasma Corrected Not corrected Not corrected Not corrected Corrected Partial Diagnosis X V II

HMWK

XII PK XI APTT IX
VIII

VII

PT
X

PT, APTT, TT - PLC - N

V II I

TT

* Hypo / dysfibrinogenemia * Heparin * Liver disease * Systemic hyperfibrinolysis

HMWK

XII PK XI APTT IX
VIII

VII

PT
X V II I

APTT, PT,TT all PLC - low

TT

* DIC - FDP - D-dimer - Fibrin monomer

HMWK

XII PK XI APTT IX
VIII

VII

PT
X
V II I

PT, APTT- TT - N PLC -

TT

Massive transfusion with stored blood

HMWK

XII PK XI APTT IX
VIII

VII

PT
X V II I

PT, APTT,TT-N PLC -

TT

Thrombocytopenia Pseudo vs True Bone marrow biopsy to differentiate production destruction

PT, APTT, TT, PLC - Normal

Factor XIII deficiency Thrombasthenia
congenital drug induced

Platelet function
BT clot retraction 1 minute platelet count aggregation

Disorders of vascular hemostasis Factor XIII - clot solubility

Tourniquet test

Asymptomatic Patient
Routine screening tests shows prolonged APTT
Inhibitor - lupus anticoagulant Factor XII deficiency Mild congenital factor deficiency

Antiphospholipid Antibody Syndrome

Criteria by Branch and Silver 1996

Clinical
Recurrent abortion Recurrent venous thrombosis Recurrent arterial thrombosis Persistent thrombocytopenia Livedo reticularis

Laboratory
IgG/IgM anticardiolipin Ab Lupus anticoagulant

Diagnosis
1 clinical + 1 lab criteria Lab result must be positive on at least 2 occasions more than 3 months apart

Lupus Anticoagulant
Kaolin clotting time
Dilute Russels viper venom time

Platelet neutralization test

Tissue thromboplastin inhibition test