Clotting Disorders

Question 1

clotting dx

Answer 1

acquired platelet dysfxn is more common than congenital

Question 2

most common causes of acquired clotting dz?

Answer 2

ASA, other NSAIDs,

uremia, alcoholism, myeloproliferative dz, hypothermia, vitamin def

Question 3

types of clotting dz

Answer 3

von willebrand

hemophilia A and B

Question 4

von Willebrand

Answer 4

autosomally inherited congenital

• characterized by reduced levels of factor V11 antigen or ristocetin cofactor
• most common inheritied bleeding dz

Question 5

what is the vWF needed for?

Answer 5

to anchor platelets to the injured vessel wall

Question 6

what is vW caused by?

Answer 6

deficiency (type 1), dysfxn (type 2) or complete absence (type 3)

Question 7

clinical features of vW dz

Answer 7

• epitaxis
• menorrhagia
• bleeding after tooth extraction
• Ecchymosis, petechiae
• gingival bleeding, traumatic oral and lip bleed
• postop bleed
• GI bleed
• hematuria
• joint bleed (type 3)
• intramuscular, deep subcutaneous or sub mucous bleed (type 3)

Question 8

dx and tx vWD

Answer 8

scree:
vWB factor anitgen
vW factor activity/ristocetin cofactor
PTT (but a normal value doesn’t exclude vWD)

Question 9

tx of vWD

Answer 9

DDAVP via desmopressin

or aminocaproic acid,

vWF containing conentrate can be used in severe episodes

avoid ASA

Question 10

what does desmopressin do?

Answer 10

stimulates the release of vWF from cells

Question 11

vit K def

Answer 11

most common acquired coagulopathies

Question 12

what happens if vit K is deficient?

Answer 12

coag is impaired

Question 13

wht can cause a vit K def?

Answer 13

malabsorption (especially w/ cystic fibrosis)

• oD of warfarin
• poor diet, liver failure, malnutrition, use of some durge (think broad specturm abx)

Question 14

what is the msot common dx results from vit K def?

Answer 14

hemorrhagic dz of the newborn! (neonates need IM vit K at birth)

Question 15

lab work up of vit K def?

Answer 15

PT is prolonged, PTT may be prolonged

fibrinogen, thrombin time, and platelet count are nomral

liver enzymes may be elevated

Question 16

Hemo. A

Answer 16

Factor 8 def!

X-linked recessive

Question 17

CF of Hemo A

Answer 17

o Symptoms are consistent with degree of clotting factor deficiency
o Mild case may go unnoticed until severe trauma
o Moderate cases bleed with trauma
o Severe cases bleed spontaneously : often noticeable by toddler stage

Question 18

lab values and dx of hemo A

Answer 18

o Prolonged aPTT: should correct to normal when mixed 1:1 with normal plasma
o PT is normal

o Based on aPTT
o Specific factor assays necessary

Question 19

tx of hemo A

Answer 19

o Prevent trauma whenever possible
o Early, appropriate replacement therapy with recombinant Factor 8
o Manage bleeding episodes at home with trained parental oversight
o Avoid circumcision in boys with family history of hemophilia

Question 20

what meds can be used to control pain in a person w/ a clotting dz?

Answer 20

o Avoid aspirin. Celecoxib or opioids may be used to control pain

Question 21

Hemo B

Answer 21

Factor 9 deficiency

X-linked recessive

“christmas dz”

Question 22

CF, and lab findings in hemo B?

Answer 22

• indistinguishable from Hemophilia A

- Laboratory Evaluation: prolonged aPTT, normal PT

Question 23

tx of Hemo B

Answer 23

o Appropriate replacement therapy (recombinant factor 9)
o Prevent trauma whenever possible
o Manage bleeding episodes at home with trained parental oversight
o Avoid circumcision in boys with family history of hemophilia

Question 24

Factor V Leiden

Answer 24

• The Leiden variant of factor V cannot be inactivated by Protein C (resistance to the protein).

Question 25

what is FVL causd by?

Answer 25

mutation. Most common hereditary hypercoagulable state.

- may be limited to those of European descent

Question 26

PP of FVL

Answer 26

o Heterozygous state increase risk of thrombosis up to 8-fold. Homozygous state increases risk 100-fold.

Question 27

tx of FVL

Answer 27

no role for family testing, no indication for primary prophylaxis

Question 28

Protein C& S def

Answer 28

• Also known as prothrombic state or thrombophilia, it is the propensity to venous thrombosis due to an abnormality in the coagulation. Reduced level of inhibitors of the coagulation cascade.

Question 29

Etiology of protein C & S def?

Answer 29

o Predisposition to recurrent venous thromboses. Like antithrombin deficiency but rather uncommon as a cause for venous thromboses.
o Protein S: commonly acquired in acute illness

Question 30

what type Protein C& S is the worst>

Answer 30

homozygous= DEATH

Question 31

pp of P C& S def?

Answer 31

o C & S are natural anticoagulants. Both are Vitamin K dependent (liver). Important in the physiological prevention of thrombosis.

Question 32

S/Sx of P C&S def?

Answer 32

o Liver disease
o Use of warfarin: warfarin induced skin necrosis (Protein C deficiency)
o Vitamin K deficiency
o Acute thrombosis
o Homozygous Protein C: presents in the neonates: purpura fulminans

Question 33

purpura fulminans?

Answer 33

is an acute, often fatal, thrombotic disorder which manifests as blood spots, bruising and discolouration of the skin resulting from coagulation in small blood vessels within the skin and rapidly leads to skin necrosis and disseminated intravascular coagulation.

Question 34

tx of P C&S def?

Answer 34

long therm warfarin w/ heterozygous C and S

Question 35

Antithrombin III def

Answer 35

, it inactivates thrombin. Reduced levels of inhibitors of the coagulation cascade.

Question 36

CF of ATIII def?

Answer 36

• Activity is markedly stimulated by Heparin. Its deficiency is manifested by recurrent venous thromboses and pulmonary embolism.

depleted in pregnancy, liver disease, nephrotic syndrome, DIC

Question 37

what are the types of ATIII D?

Answer 37

Homozygous = Death; Heterozygous = Asymp

Question 38

PP of ATIII

Answer 38

deficiency allows unopposed conversion of fibrinogen to fibrin.

Question 39

tx of ATIII

Answer 39

anticoags