Bleeding disorders Flashcards
difference between the coagulation effect from a medicine like ASA vs a medicine like coumadin
- ASA - platelet toxin (resists clotting)
2. Coumadin - inhibits clotting
examples of dzs that effect primary hemostasis
ITP TTP HUS DIC VwFD
briefly describe primary hemostasis
platelets form a plug
aggregate send
ADP and thromboxane A2 which attract other platelets
PT and PTT unaffected because these are clotting factors
dzs of primary hemostasis typically involve sxs like
petechiae and mucotaneous bleeding
oral
GI
menorrhagia
dzs that affect secondary hemostasis
hemophilia
DIC
vWD
sxs associated with dzsz of secondary hemostasis
deep delayed bleeding
hemarhtrosis seen with bleeding into the joints and delayed bleeding after surgery
PTT measures what factors
12
11
9
8
major intrinsic
alos
5
2
1
heparin DIC vWD heophilia
PT measures
7 and 10
also
1
2 5
warfarin
vitamin K deficiency
DIC
Hereditary Hemorrhagic Telangiectasia shows what type of inheritance pattern
AD
Osler, Weber, Rendu
what is the pathophys behind heridditary hemorrhagic talangictasia
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
when do you usually see hereditary hemorrhagic talangictasia
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
vascular disorder that effects all collagen sturctures
ehler danlos syndrome
sxs of ehler danlos
” Easy bruising, hyperelasticity of skin, hypermobility of joints, weakness of tissues
fibrilin 1 binds TGF beta creating inflammatory effects that damage the aorta and heart
marfans
this vascular disorder is associated with AVM in the brain lungs and liver
HHT
thrombocytopenia can result from
decreased bone marrow production
sequestration from the spleen
increased platelet destruction
features of a platelet d/o
normal PT and PTT
prlonged bleeding time
spontaneous bleeding
decrease platelets
causes of thrombocytopenia
- aplastic-bad bone marrow
- leukemia-bone marrow infultration
- ineffective thrombopoiesis (B12/Folate def)
- increased destruction
- infection –> will lower
- consumption (DIC)
ITP is characterized by this imnune reaction
IgG Vs GPIIb/IIIA
MCC thombocytopenia in children
pentad of TTP
" Thrombocytopenia " Microangiopathic anemia " Neurologic changes " Renal failure " Fever
Adams 13
FAR to TAN
splendid
plasma TV
pathophys of TTP
” Extensive microscopic clotting in small blood vessels by failure to cleave vWF polymers (enzyme ADAMTS13). Shear stress on RBC –> hemolysis
sxs associated with TTP
” Darkening of urine from hemolytic anemia
“ Small clots lead to widespread areas of ischemia (multi organ damage)
“ N/V from gut ischemia
this platelet disorder can occur with recent use of heparin
ii. IgG mediated complexes destroy platelets
iii. Unexplained platelet loss or sudden bleeding
iv. Withdraw agents, get assay for previous hx; get platelet counts
MCC of AKI in children
HUS
what is hemolytic uremic syndrome
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
difference between HUS and TTP
HUS= AKI and RF no fever
TTP= mental sxs
MC initiating facotrs of HUS
Diarrhea and URI are MC inciting factors
suspect HUS in children with
AKI and diarrhea prodrome
dx with HUS
increase in BUN and Creatinin
and of course thrombocytopenia
what dxs would we expect to see associated with the microangiopathic anemia in HUS and TTP
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
broad categories of coagulation disorders
production
inhibition
consumptaion
and liver dz
Factor VIII Deficiency
hemophilia A
MCC of hemophilia
pathophys of hemophilia A
required for activation of factor X by intrinsic pathway
liver synthesis
bound to subendothelium by vWF
inheritance characteristics of hemophilia
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
presentation of hem A
late rebleeding
hemarthroses
bruise easily
massive hemorrhage
how would you dx hem A
PTT
low levels VIII
ALSO SEEN WITH bt
TX for hem A
o Factor VIII replacement
o Desmopressin Acetate for smaller bleeds (it is vasoconstricting)
christmas disease
hem B caused by factor IX deficiency
sxs of xmas dz
indistinguishable form hem A late rebleeding hemarthroses bruise easily massive hemorrhage
need factor assay
what reverses heparin
- Protamine sulfate reverses
what reverses warfarin
Reversed by administration of Vit K!
iv. ASA (De Rosa’s favorite) í Acetylsalicylic acid
works by
- COX 1 and 2 inhibitor
- Inhibits prostaglandin and thromboxane production
- Blocking thromboxane A2 production prevents platelet aggregation
what increases clotting
i. Vit K, FFP (fresh frozen plasma), factor replacement, antifibrinolytics, desmopressin
