Clotting Disorders Flashcards
clotting dx
acquired platelet dysfxn is more common than congenital
most common causes of acquired clotting dz?
ASA, other NSAIDs,
uremia, alcoholism, myeloproliferative dz, hypothermia, vitamin def
types of clotting dz
von willebrand
hemophilia A and B
von Willebrand
autosomally inherited congenital
- characterized by reduced levels of factor V11 antigen or ristocetin cofactor
- most common inheritied bleeding dz
what is the vWF needed for?
to anchor platelets to the injured vessel wall
what is vW caused by?
deficiency (type 1), dysfxn (type 2) or complete absence (type 3)
clinical features of vW dz
- 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)
dx and tx vWD
scree:
vWB factor anitgen
vW factor activity/ristocetin cofactor
PTT (but a normal value doesn’t exclude vWD)
tx of vWD
DDAVP via desmopressin
or aminocaproic acid,
vWF containing conentrate can be used in severe episodes
avoid ASA
what does desmopressin do?
stimulates the release of vWF from cells
vit K def
most common acquired coagulopathies
what happens if vit K is deficient?
coag is impaired
wht can cause a vit K def?
malabsorption (especially w/ cystic fibrosis)
- oD of warfarin
- poor diet, liver failure, malnutrition, use of some durge (think broad specturm abx)
what is the msot common dx results from vit K def?
hemorrhagic dz of the newborn! (neonates need IM vit K at birth)
lab work up of vit K def?
PT is prolonged, PTT may be prolonged
fibrinogen, thrombin time, and platelet count are nomral
liver enzymes may be elevated
Hemo. A
Factor 8 def!
X-linked recessive
CF of Hemo A
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
lab values and dx of hemo A
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
tx of hemo A
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
what meds can be used to control pain in a person w/ a clotting dz?
o Avoid aspirin. Celecoxib or opioids may be used to control pain
Hemo B
Factor 9 deficiency
X-linked recessive
“christmas dz”
CF, and lab findings in hemo B?
- indistinguishable from Hemophilia A
- Laboratory Evaluation: prolonged aPTT, normal PT
tx of Hemo B
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
Factor V Leiden
- The Leiden variant of factor V cannot be inactivated by Protein C (resistance to the protein).
what is FVL causd by?
mutation. Most common hereditary hypercoagulable state.
- may be limited to those of European descent
PP of FVL
o Heterozygous state increase risk of thrombosis up to 8-fold. Homozygous state increases risk 100-fold.
tx of FVL
no role for family testing, no indication for primary prophylaxis
Protein C& S def
- 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.
Etiology of protein C & S def?
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
what type Protein C& S is the worst>
homozygous= DEATH
pp of P C& S def?
o C & S are natural anticoagulants. Both are Vitamin K dependent (liver). Important in the physiological prevention of thrombosis.
S/Sx of P C&S def?
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
purpura fulminans?
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.
tx of P C&S def?
long therm warfarin w/ heterozygous C and S
Antithrombin III def
, it inactivates thrombin. Reduced levels of inhibitors of the coagulation cascade.
CF of ATIII def?
- 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
what are the types of ATIII D?
Homozygous = Death; Heterozygous = Asymp
PP of ATIII
deficiency allows unopposed conversion of fibrinogen to fibrin.
tx of ATIII
anticoags
