Bleeding Disorders--Krafts Flashcards
Platelet bleeding is characterized by…
spontaneous bleeding
petichaie
Factor bleeding is characterized by…
big bleeds after trauma
esp. into deep joints
*lots of bleeding, not like petichae (like platelet bleeding)
Petichiae are characteristic of…
platelet bleeding
Big, joint space bleeding is characteristic of…
factor bleeding
Type 1 vWD problem
decreased vWF
(75%)
Type 2 vWD problem…
abnormal vWF
(25%)
Type 3 vWD problem…
no vWF
(5%)
Sx of vWD
mucosal bleeding
deep joint bleeding in severe cases
vWD
Bleeding time
PTT
INR
[vWF]
Platelet aggregation studies
Bleeding time: prolonged
PTT: prolonged “corrects” w/ mixing study
INR: normal
[vWF]: normal, except type 2
Platelet aggregation studies: abnormal
vWF binds which GP?
GPIb
GPIa
GPIb
GPIIb/IIIa
GPIa: collagen
GPIb: vWF
GPIIb/IIIa: fibrinogen
Tx of vWD
cryoprecipitate (contains vWF and VIII)
Hemophilia inheritance pattern
X-linked recessive
Most common factor deficiency
Hemophilia A
Hemophilia A deficiency
Factor VIII
(levels decreased)
Hemophilia A Sx
“Factor bleeding”
- deep joint bleeding
- prolonged bleeding after dental work
*rarely, mucosal hemorrhage
Hemophilia A
INR, TT, platelet count, bleeding time
PTT
Factor VIII assays: abnormal
DNA studies: abnormal
INR, TT, platelet count, bleeding time: normal
PTT: prolonged (corrects with mixing study)
Factor VIII assays: abnormal
DNA studies: abnormal
Tx of Hemophilia A
Factor VIII replacement
Hemophilia B pathology
Factor IX deficiency
Bernard-Soulier Syndrome
pathology
abnormal Ib (vWF receptor)
Bernard-Soulier Syndrome
severe bleeding
big platelets
Glanzmann Thrombasthenia
pathology
GPIIb/IIIa deficiency
no aggregation
Gray platelet syndrome
pathology
no alpha granules
mild bleeding
Delta granule deficiency
pathology
absent delta granules
may be part of another syndrome
Why do you bleed in DIC?
use up all platelets
use all clotting factors
What type of anemia is associated with DIC?
microangiopathic hemolytic anemia
What sets off DIC?
initiation of coagulation
(it’s not a platelet problem)
Big causes of DIC
malignancy
OB complications
sepsis
trauma
MOST cases acrynym
DIC
INR, PTT, TT
FDPs
Fibrinogen
INR, PTT, TT: prolonged
FDPs: increased
Fibrinogen: decreased
Idiopathic Thrombocytopenic Purpura
pathology
antiplatelet antibodies
GPIIb/IIIa or Ib
splenic macrophages eat platelets
Chronic
vs
Acute
Idiopathic Thrombocytopenic Purpura
Chronic: big problem–bleeding into brain
Acute: abrupt, follows viral illness (usually children)
Labs in idiopathic thrombocytopenic purpura
thrombocytopenia
many/bigger megas in BM
bigger platelets in smear
TTP
pathology
ADAMTS13 deficiency
ADAMTS13 normally cleaves new, large vWF to less active bits
if they are not able to be cleaved, then you get a lot of platelet aggregation –> little clots everywhere
TTP
pentad
MAHA
thrombocytopenia
fever
neurologic defects
renal failure
Why do you get neurlogic defects in TTP?
many little clots that end up going to the brain
Two things you will have in TTP
microangiopathic hemolytic anemia
thrombocytopenia
+ neurological deficits
TTP Tx
acquired: plasmapheresis
hereditary: plasma infusions
Hemolytic Uremic Syndrome
pathology
E. coli infections
- most likely form toxin
- endothelial damage (platelet activation)
*this is the reason that E. coli outbreak patients are hospitalized
Hemolytic Uremic Syndrome
clinical findings
blood diarrhea
then renal failure
5% fatality rate
Tx of hemolytic uremic syndrome
NOT ABX!!
supportive care
dialysis
