Bleeding disorders

Question 1

difference between the coagulation effect from a medicine like ASA vs a medicine like coumadin

Answer 1

1. ASA - platelet toxin (resists clotting)

2. Coumadin - inhibits clotting

Question 2

examples of dzs that effect primary hemostasis

Answer 2

ITP
TTP
HUS
DIC
VwFD

Question 3

briefly describe primary hemostasis

Answer 3

platelets form a plug
aggregate send
ADP and thromboxane A2 which attract other platelets

PT and PTT unaffected because these are clotting factors

Question 4

dzs of primary hemostasis typically involve sxs like

Answer 4

petechiae and mucotaneous bleeding

oral
GI
menorrhagia

Question 5

dzs that affect secondary hemostasis

Answer 5

hemophilia
DIC
vWD

Question 6

sxs associated with dzsz of secondary hemostasis

Answer 6

deep delayed bleeding

hemarhtrosis seen with bleeding into the joints and delayed bleeding after surgery

Question 7

PTT measures what factors

Answer 7

12
11
9
8

major intrinsic
alos

5
2
1

heparin DIC vWD heophilia

Question 8

PT measures

Answer 8

7 and 10

also
1
2 5

warfarin
vitamin K deficiency
DIC

Question 9

Hereditary Hemorrhagic Telangiectasia shows what type of inheritance pattern

Answer 9

AD

Osler, Weber, Rendu

Question 10

what is the pathophys behind heridditary hemorrhagic talangictasia

Answer 10

defects of TGF beta-1 effect formation of connective tissue necessary for BV formation

results in knots of blood vessels b/c lack of normal basement membrane

Question 11

when do you usually see hereditary hemorrhagic talangictasia

Answer 11

during adolescents Mucosla and during adulthood they are cutaneous on the muscosal membranes (nasal and orolabial), face, and distal extremities.

seen with reoccurent epistaxis and GIB

Question 12

vascular disorder that effects all collagen sturctures

Answer 12

ehler danlos syndrome

Question 13

sxs of ehler danlos

Answer 13

” Easy bruising, hyperelasticity of skin, hypermobility of joints, weakness of tissues

Question 14

fibrilin 1 binds TGF beta creating inflammatory effects that damage the aorta and heart

Answer 14

marfans

Question 15

this vascular disorder is associated with AVM in the brain lungs and liver

Answer 15

HHT

Question 16

thrombocytopenia can result from

Answer 16

decreased bone marrow production

sequestration from the spleen

increased platelet destruction

Question 17

features of a platelet d/o

Answer 17

normal PT and PTT
prlonged bleeding time
spontaneous bleeding
decrease platelets

Question 18

causes of thrombocytopenia

Answer 18

1. aplastic-bad bone marrow
2. leukemia-bone marrow infultration
3. ineffective thrombopoiesis (B12/Folate def)
4. increased destruction
5. infection –> will lower
6. consumption (DIC)

Question 19

ITP is characterized by this imnune reaction

Answer 19

IgG Vs GPIIb/IIIA

MCC thombocytopenia in children

Question 20

pentad of TTP

Answer 20

"	Thrombocytopenia
"	Microangiopathic anemia
"	Neurologic changes
"	Renal failure
"	Fever

Adams 13
FAR to TAN
splendid
plasma TV

Question 21

pathophys of TTP

Answer 21

” Extensive microscopic clotting in small blood vessels by failure to cleave vWF polymers (enzyme ADAMTS13). Shear stress on RBC –> hemolysis

Question 22

sxs associated with TTP

Answer 22

” Darkening of urine from hemolytic anemia
“ Small clots lead to widespread areas of ischemia (multi organ damage)
“ N/V from gut ischemia

Question 23

this platelet disorder can occur with recent use of heparin

Answer 23

ii. IgG mediated complexes destroy platelets
iii. Unexplained platelet loss or sudden bleeding
iv. Withdraw agents, get assay for previous hx; get platelet counts

Question 24

MCC of AKI in children

Answer 24

HUS

Question 25

what is hemolytic uremic syndrome

Answer 25

similar to TTP but associated with E. Coli toxins and chemotherapeutic agents these exotoxins cause platelet activation and lead to damage in the vascular endothelieum that eventually leads to paltelet deletion thrombocytopenia and damage to to

Question 26

difference between HUS and TTP

Answer 26

HUS= AKI and RF no fever TTP= mental sxs

Question 27

MC initiating facotrs of HUS

Answer 27

Diarrhea and URI are MC inciting factors

Question 28

suspect HUS in children with

Answer 28

AKI and diarrhea prodrome

Question 29

dx with HUS

Answer 29

increase in BUN and Creatinin and of course thrombocytopenia

Question 30

what dxs would we expect to see associated with the microangiopathic anemia in HUS and TTP

Answer 30

``` we would expect to see reticulocytes increase in the peripheral smear scistocytes (bile/fragmented cells) increase in LDH increase in indirect bili decrease in haptoglobin ``` coombs negative and spleenomegaly

Question 31

broad categories of coagulation disorders

Answer 31

production inhibition consumptaion and liver dz

Question 32

Factor VIII Deficiency

Answer 32

hemophilia A MCC of hemophilia

Question 33

pathophys of hemophilia A

Answer 33

required for activation of factor X by intrinsic pathway liver synthesis bound to subendothelium by vWF

Question 34

inheritance characteristics of hemophilia

Answer 34

sxs usually occur before 18 and it is x linked recessive 30% random mutation so Fhx Can't rule out 10% have normal facotr levels but are inhibited by AB

Question 35

presentation of hem A

Answer 35

late rebleeding hemarthroses bruise easily massive hemorrhage

Question 36

how would you dx hem A

Answer 36

PTT low levels VIII ALSO SEEN WITH bt

Question 37

TX for hem A

Answer 37

o Factor VIII replacement | o Desmopressin Acetate for smaller bleeds (it is vasoconstricting)

Question 38

christmas disease

Answer 38

hem B caused by factor IX deficiency

Question 39

sxs of xmas dz

Answer 39

``` indistinguishable form hem A late rebleeding hemarthroses bruise easily massive hemorrhage ``` need factor assay

Question 40

what reverses heparin

Answer 40

6. Protamine sulfate reverses

Question 41

what reverses warfarin

Answer 41

Reversed by administration of Vit K!

Question 42

iv. ASA (De Rosa's favorite) í Acetylsalicylic acid works by

Answer 42

9. COX 1 and 2 inhibitor 10. Inhibits prostaglandin and thromboxane production 11. Blocking thromboxane A2 production prevents platelet aggregation

Question 43

what increases clotting

Answer 43

i. Vit K, FFP (fresh frozen plasma), factor replacement, antifibrinolytics, desmopressin