Please enable JavaScript.
Coggle requires JavaScript to display documents.
Coagulation Disorders (Thrombophilia, APL) (THROMBOPHILIA (INHERITED…
Coagulation Disorders (Thrombophilia, APL)
-
THROMBOPHILIA
- tendency to venous or arterial thrombosis
-
other symptoms
Thrombosis of cns veins (prothrombin gene mutation)
Thrombosis of abdominal veins: hepatic portal vein, hepatic veins ( budd-chiarie syndrome)
Antenatal complications: placenta abruption, miscarriages (ii i iii term) >3 x, dead foetus birth
Aseptic / ischemic bone necrosis
Arterial thrombosis (stork) –aps
Skin necrosis in few first days of oral anticoagulant treatment –deficiency of protein c and s
INHERITED
-
etiology
Rare
- tafi activity -increase
- pai activity -increase
- lipoprotein a concentration-increase
- propably–deficiency of: xii, prekalikreine, kininogen
- dysfibrynogenemia (chapel hill, marburg, haifa)
- homozygosity type of homocyteinuria:
syntase of b-cystationin, mthfr mutations
- plasminogen deficiency
- viii f -increase
common
- Factor V - leiden ( A1691G mutations)
- prothrombin gene mutation
- Protein C deficiency
- Protein S deficiency
- AT deficiency
TESTS
Physical examination
Coagulation/ D-dimers
Doppler USG
Angio-ck / scyntigraphy -lungs
ECG /gasometry
MRI CNS–Angio MRI
- SPECIFIC TESTS:
•activated protein c resistance (positive in 95% of leiden mutation )
•prothrombin gene mutation
•homocysteine in serum
•activity of protein c (<50%)
•activity of s -proteins (<50%)
•at activity (<60%)
•VIII activity (>200% = iu/dl)
•fibrinogen, dysfibrynogenemia
•IX , XI
TREATMENT
HEPARIN
IF NECESSARY rt-PA (ACTYLISE) –PULMONARY EMBOLISM, STORK
AFTER SOME DAYS ORAL ANTICOAGULANT (INR 2,5-3)
-