coagulopathies

Question 1

generally speaking whenever there is abnormal platelets whats the presentation like ?

Answer 1

ususally presents as mucosal bleeding, skin bleeding and petechia

Question 2

generally what is the presentation when it comes to abnormal coagulation factors ?

Answer 2

joint bleeding , deep tissue bleeding

Question 3

what is the mode of inheritance associated with hemophilia and what are the deficient factors ?

Answer 3

hemophilia A : factor 8
hemophilia B : factor 9 also called christmas disease
hemophilia C: factor 11

Question 4

what is the presentation of hemophilia ?

Answer 4

present with spontaneous and easy bleeding of the joints

Question 5

what are the lab results associated factor with hemophilia ?

Answer 5

everything is normal except the PTT is prolonged

Question 6

what is the treatment for hemophilia ?

Answer 6

factor 10 or factor 9 replacement
desmopressin
aminocaproic acid

Question 7

when is desmopressin used ?

Answer 7

mild hemophilia A , it increased vWF and factor 8 levels

Question 8

what are the side effects of desmopressin ?

Answer 8

flushing and headache due to vasodilator effect

Question 9

what are the other uses associated with desmopressin use ?

Answer 9

von Willebrand disease
central diabetes insipidus
bedwetting in children

Question 10

what are the associations with coagulation factor inhibitors and clotting factors ?

Answer 10

inhibit or increase clearance of clotting factors , inhibitors of factor 8 is the most common

Question 11

what are the associations with coagulation factor inhibitors ?

Answer 11

malignancy
pregnancy
autoimmune disorders

Question 12

what is the treatment for coagulation factor inhibitors ?

Answer 12

prednisone

Question 13

how can we differentiate between coagulation factor inhibitors and hemophilia A ?

Answer 13

differentiated by using a mixing study if the PTT corrects itself then it was a factor deficiency meaning it was one of the hemophilias
if the PTT stays prolonged then it is a coagulation factor inhibitor most commonly lupus anticoagulant

Question 14

what disorder is associated with factor 12 deficiency ?

Answer 14

a deficiency in hageman factor is asymptomatic - no bleeding disorder

Question 15

what are the classic cases assocated with hemophilia ?

Answer 15

a child with recurrent joint bleeding and has a relative who had the same thing and died early

or a nenonate that suffers from excessive bleeding in circumscion

Question 16

if a patient with hemophilia A is being treated byt the PTT stays prolonged what is the next best step ?

Answer 16

check for factor 8 inhibitors with mixing study

Question 17

what is the problem with vitamin k defeciency ?

Answer 17

there is a defeciency in vitamin k dependant factors , factor 2, 7 , 9 , 10

Question 18

what are the lab findings associated with vitamin K deficiency ?

Answer 18

elevated PT/INR
normal bleeding time

Question 19

what are the classic cases associated with vitamin K deficiency ?

Answer 19

1) neonate that has bleeding from their umbilical stump

2) adult who has been on wide spectrum antibiotic for a long time and now needs warfarin , warfarin inhibits vitamin K function

3) exposure to rat poison which has a warfarin like effect

Question 20

what are the common causes of vitamin K deficiency ?

Answer 20

warfarin use
antibiotic use for a long time
newborns
malabsorption ( vitamin K is fat soluble)
large volume of blood transfusion

Question 21

what is the treatment for vitamin K deficiency after large blood transfusion ?

Answer 21

fresh frozen plasma if there is active bleeding

Question 22

why is there coagulopathy in advanced liver disease ?

Answer 22

decreased synthesis of clotting factors , except factor 8 , associated with thrombocytopenia

Question 23

what test is more sensitive to detect coagulopathy associated with liver disease ?

Answer 23

PT

Question 24

what lab finding is the first to fall in vitamin K deficiency ?

Answer 24

PT