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 - o APTT, TT, PLC - N
TT
* Factor VII deficiency * Anticoagulant therapy
HMWK
XII PK XI APTT IX
VIII
VII
PT
X V II I
APTT - o 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
Corrected No correction Corrected
VIII IX 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 q 2N - q affinity for FVIII 3 - severe deficiency of vWF
HMWK
XII PK XI APTT IX
VIII
VII
PT
X
PT, APTT - o 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 - o PLC - N
V II I
TT
* Hypo / dysfibrinogenemia * Heparin * Liver disease * Systemic hyperfibrinolysis
HMWK
XII PK XI APTT IX
VIII
VII
PT
X
APTT, PT,TT all o PLC - low
V II I
TT
* DIC - FDP - D-dimer - Fibrin monomer
HMWK
XII PK XI APTT IX
VIII
VII
PT
X V II I
PT, APTT- o TT - N PLC - q
TT
Massive transfusion with stored blood
HMWK
XII PK XI APTT IX
VIII
VII
PT
X V II I
PT, APTT,TT-N PLC - q
TT
Thrombocytopenia Pseudo vs True Bone marrow biopsy to differentiate q production o 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
