disorders of clotting Flashcards
what is cocktail purpura
happens in people who drink gin and tonic
tonic water has quinine which causes platelts to clump and be removed from the body
-petechiae may show up the next day
what is the normal PT
10-13 seconds
PT prolonged with what deficicny and what drug
factors II, V, VII, X and fibrinogen
warfarin or dicoumarol
normal PTT time
25-40
PTT prolonged in what deficiency and what drug
factors VIII, IX, XI, XII
heparin
what does Thrombin time (TT) assess for
deficiency or abnormalities of fibrinogen
most common qualitative platetelet defect
in pts taken aspirin or NSAIDs
treatment of DIC
correction of underlynig disorder- sepsis, bowel obstruction etc
supporive care- platelet and factor replacement
thrombotic thrombocytopenic purpura
add one more symptom and it gives you what
characterized by purpura, microangiopathic hemolytic anemia, fluctuating neurological signs, renal dysfunction and febrility anad thrombocytoenia under 50,000
add renal failure = HUS
helmet cells known as waring blender effect
TTP
-hyaline thrombi occlude capillaries of virtually every organ in the body
cause of acquired TTP
autoantibodies directed at ADAMTS13
linked to infection, malignancy, immune disorders
treatment for TTP
plasmapheresis
treatment for VWD
cryoprecipitate –>replaces vWF
DDAVP–> causes relase of vWF from endothelium
antifibrinolytic agents like aminocaproic acid
PT with deficiency of vitamin K dep factors
prolonged
hereditary hemorrhagic telangiectasia
only endothelial syndrome associated with hemostatic complications
-caused by thinning of vessel walls with telangiectatic formations, AV malformations, and aneurysmal dilations throughout the body
HHT inheritence and defect
autosomal dominant
defect in gene coding for endoglin on chrom 9
clinical features of HHT
telangiectasis on tip of tongue and lip mainly
bleeding to mild or inapparent trauma
-epistaxis is the most frequent symptom
course and treatment of HHT
usually benign clinical course, recurrent bleeds frequent but death rare
surgery and laser photoablation of telangiectasias of some value but care must be used in selecting site
antithrombin III deficiency clinical presentation
variable from minimal symptomatology to early death from recurrent pulmonary emboli
50% of affected pts have DVT and or PE by age 30
AT III diagnosis
less than 50% of normal activity of AT III levels in serum
treatment for AT III deficiency
prophylactic treatment with anticoags
pts with DVT need heparin but much higher doses required
AT III replacement therapy is available for known deficient pts
protein C and S function
S is cofactor for C which inactivates V, and VIII
what do you give pts with protein C and S deficiency
warfarin to decrease the risk of thromboembolic disaeae
-beware that warfarin can also suppress these factors causing a hypercoagulable state
factor V leiden and treatment
abnormalitiy of factor V at binding site for activated protein C
no prior DVT just moniter
prior episodes then consider lifelong anticoagulation
prothrombin 20210
increaed activity for prothrombin bc of mutation so it isn’t deactivated
risk of thrombosis is high
-same treatment as factor V
antiphospholipid syndrome
aka anticardiolipin antibody syndrome
lupus anticoagulant
false postivtve VDRL antibody syndrome
clincial clues suggesting an inherited hypercoagulable disorder
thrombosis at young age under 50
multiple thromboses episodes
thrombosis in an unusual site
family history of thrombosis
characterized by venous and arterial thrombsois
recurrent spontaneuous pregnancy loss
thrombocytopenia
neuropsychiatric manifestaions
antiphospholipid antibody syndrome
what events most common in antiphospholipid antibody syndrome
how to treat
cerebrovascular events most common arterial thrombotic complication with stroke and TIA and dementia of retinal artery occlusion
placental vessel thrombosis leads to pregnacny loss and fetal growth retardation
-treat with warfarin
what causes false postitive VDRL antibody syndrome
antiphos syndrome when testing for neurosyphillis
associated features of APS
CT disease
prolonged PTT, fails to correct with mixing
valvular heart disease in some
coronary artery disease
diagnosis; 3 test APS
prolonged PTT
lack of correction in mixing
neutralization of inhibitor with excess phospholipid
confiramtion test APS
DRVVT
treatment for APS
diagnosis confirmation
those with history of thromboembolic disease: lifelong anticoagulation
need multiple postiive tests over 3-12 month period to make diagnosis
what can help reduce thromboembolism in pts with APS and SLE
hyroxychloroquine
most common cause of hereditary hypercoagubility are
factor V leiden and prothrombin gene mutation
what does a hypercoag panal include
factor v and prothrombin test
and also protein C and S test
won’t include APS antibody and lupus anticoagulant
treatment of APS during pregnancy
SC heparin
