disorders of clotting

Question 1

what is cocktail purpura

Answer 1

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

Question 2

what is the normal PT

Answer 2

10-13 seconds

Question 3

PT prolonged with what deficicny and what drug

Answer 3

factors II, V, VII, X and fibrinogen

warfarin or dicoumarol

Question 4

normal PTT time

Answer 4

25-40

Question 5

PTT prolonged in what deficiency and what drug

Answer 5

factors VIII, IX, XI, XII

heparin

Question 6

what does Thrombin time (TT) assess for

Answer 6

deficiency or abnormalities of fibrinogen

Question 7

most common qualitative platetelet defect

Answer 7

in pts taken aspirin or NSAIDs

Question 8

treatment of DIC

Answer 8

correction of underlynig disorder- sepsis, bowel obstruction etc

supporive care- platelet and factor replacement

Question 9

thrombotic thrombocytopenic purpura

add one more symptom and it gives you what

Answer 9

characterized by purpura, microangiopathic hemolytic anemia, fluctuating neurological signs, renal dysfunction and febrility anad thrombocytoenia under 50,000

add renal failure = HUS

Question 10

helmet cells known as waring blender effect

Answer 10

TTP

-hyaline thrombi occlude capillaries of virtually every organ in the body

Question 11

cause of acquired TTP

Answer 11

autoantibodies directed at ADAMTS13

linked to infection, malignancy, immune disorders

Question 12

treatment for TTP

Answer 12

plasmapheresis

Question 13

treatment for VWD

Answer 13

cryoprecipitate –>replaces vWF
DDAVP–> causes relase of vWF from endothelium
antifibrinolytic agents like aminocaproic acid

Question 14

PT with deficiency of vitamin K dep factors

Answer 14

prolonged

Question 15

hereditary hemorrhagic telangiectasia

Answer 15

only endothelial syndrome associated with hemostatic complications
-caused by thinning of vessel walls with telangiectatic formations, AV malformations, and aneurysmal dilations throughout the body

Question 16

HHT inheritence and defect

Answer 16

autosomal dominant

defect in gene coding for endoglin on chrom 9

Question 17

clinical features of HHT

Answer 17

telangiectasis on tip of tongue and lip mainly
bleeding to mild or inapparent trauma
-epistaxis is the most frequent symptom

Question 18

course and treatment of HHT

Answer 18

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

Question 19

antithrombin III deficiency clinical presentation

Answer 19

variable from minimal symptomatology to early death from recurrent pulmonary emboli
50% of affected pts have DVT and or PE by age 30

Question 20

AT III diagnosis

Answer 20

less than 50% of normal activity of AT III levels in serum

Question 21

treatment for AT III deficiency

Answer 21

prophylactic treatment with anticoags
pts with DVT need heparin but much higher doses required
AT III replacement therapy is available for known deficient pts

Question 22

protein C and S function

Answer 22

S is cofactor for C which inactivates V, and VIII

Question 23

what do you give pts with protein C and S deficiency

Answer 23

warfarin to decrease the risk of thromboembolic disaeae

-beware that warfarin can also suppress these factors causing a hypercoagulable state

Question 24

factor V leiden and treatment

Answer 24

abnormalitiy of factor V at binding site for activated protein C

no prior DVT just moniter
prior episodes then consider lifelong anticoagulation

Question 25

prothrombin 20210

Answer 25

increaed activity for prothrombin bc of mutation so it isn't deactivated risk of thrombosis is high -same treatment as factor V

Question 26

antiphospholipid syndrome

Answer 26

aka anticardiolipin antibody syndrome lupus anticoagulant false postivtve VDRL antibody syndrome

Question 27

clincial clues suggesting an inherited hypercoagulable disorder

Answer 27

thrombosis at young age under 50 multiple thromboses episodes thrombosis in an unusual site family history of thrombosis

Question 28

characterized by venous and arterial thrombsois recurrent spontaneuous pregnancy loss thrombocytopenia neuropsychiatric manifestaions

Answer 28

antiphospholipid antibody syndrome

Question 29

what events most common in antiphospholipid antibody syndrome how to treat

Answer 29

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

Question 30

what causes false postitive VDRL antibody syndrome

Answer 30

antiphos syndrome when testing for neurosyphillis

Question 31

associated features of APS

Answer 31

CT disease prolonged PTT, fails to correct with mixing valvular heart disease in some coronary artery disease

Question 32

diagnosis; 3 test APS

Answer 32

prolonged PTT lack of correction in mixing neutralization of inhibitor with excess phospholipid

Question 33

confiramtion test APS

Answer 33

DRVVT

Question 34

treatment for APS | diagnosis confirmation

Answer 34

those with history of thromboembolic disease: lifelong anticoagulation need multiple postiive tests over 3-12 month period to make diagnosis

Question 35

what can help reduce thromboembolism in pts with APS and SLE

Answer 35

hyroxychloroquine

Question 36

most common cause of hereditary hypercoagubility are

Answer 36

factor V leiden and prothrombin gene mutation

Question 37

what does a hypercoag panal include

Answer 37

factor v and prothrombin test and also protein C and S test won't include APS antibody and lupus anticoagulant

Question 38

treatment of APS during pregnancy

Answer 38

SC heparin