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28 Cards in this Set

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Tx for vWF Dz
demospression (DDAVP)
Tx for Factor 8 deficiency (Hemophilia A)
desmopression (DDAVP)
Tx for deficiency of all other coagulation factors besides Factor 8 and 9
FFP;
except hypofibrinogenemias--Tx with cryoprecipitate
what is liver's role in hemostasis
1. synthesize all hemostate proteins
2. cler activate protein adn FDP (Fibrin Degradation Products)
3. produce Bile (req for Vit K absorption, which is needed to syn Factors 2,7,9,10,Protein C and S)
hemostatic effects of liver Dz
1. decreased bile-->dec Vit K abs-->decreased production of Coag Factors (2,7,9,10) & Coag factor inhibitors (AT, Protein C and S)
2. decreased clearance of FDP (fibrin degradation products)--assoc w/DIC
Vit K is what
Vit K=fat soluble quinones derived from plants (K1) or microorganisms (K2)
what is the Vit K cycle
red-ox cycle that brings about gamma-carboxylation of glutamic acid residues in the precursors of Factors 2,7,9, and 10, as well as protein C and S in the liver
-->gamma-carboxylaton confers ability of these factors to bind Ca2+, which is essential for their in vivo activation
Vit K deficiency causes what problems
1. HDN
2. Malabsorption Syndromes (caused by biliary obstruction)
3. Intestinal sterilization (loss of K2 from enteric bacteria)
what meds can cause bleeding
(anticoagulants)
1. Warfarin (oral)
2. Heparin
what causes thrombocytopenia
1. decreased production by BM (<--decreased megakaryocytes)
2. ineffective thrombopoiesis
(i.e. megaloblastic anemia)
(abnl megakaryocytes)
3. inc platelet destruction (megakaryocytes increased to replace destroyed platelets; e.g. DIC, HUS, TTP)
4. loss of platelets (due to massive bleeding)
5. Abnormal sequestration of platelets (into spleen) (megakaryocytes increased to replace platelets)
Tx Thrombocytopenia
1. treat underlying D/O
2. platelet transfusions
3. splenectomy
Sx of thrombocytosis
thrombosis (platelet>1 million) OR bleeding
Sx of von Willebrand Dz
bleeding in skin, mucus membranes, GI, and urinary tract
what technique used to classify VW Dz
multimeric analysis
classification of VW Dz
Type 1-Heterozygous, mild
Type 2-Dysfxn'al vWF mcs, subtypes a,b,m,n; resembles mild hemophilia b/c although Factor 8 is normal, defect on vWF where Factor 8 binds, so can't bind

Type 3-homozygous or double heterozygous, severe
is the mcs in Type 2 vWD normal or abnl?
abnl
pathogenesis of vWD
a. defect in vWF subunit structure
b. defective platelet-vWF interaxn
how Tx vWD
vWF replacement
Bernard-soulier syndrome
1. platelet membrane defect: GP1b-9 complex absent
2. decreased aggregation in response to vWF (+ristocetin)
3. nl aggregation to ADP, collagen, EPI, thrombin
thrombasthenia
1. platelet membrane defect: GP2,3 absent
2. decreased agg in response to ADP, collagen, EPI, thrombin
3. nl agg to vWF (+ristocetin)
what are the inherited Qualittive Platelet D/O
1. vWD
2. Bernard-Soulier
3. Thrombasthenia
4. Storage Pool D/O
Storage Pool D/O involves what?
dec platelet agg b/c:
1. defective SERO uptake into platelets
2. defective SERO release from platelets
acquired qualitive platelet D/O?
**Uremia: poor platelet fxn b.c inhibited by retained organic acids (guanidinosuccinic acid)
-drug (aspirin)
-liver dz
-myeloprolif d/o
-paraproeinemias
Pt w/renal failure and GI bleed. Platelet transfusion doesn't work. What do you do?
give them dialysis to remove organic acids! (Uremia: kidney can't clear organic acids (guanidinosuccinnic acid)-->inhibits platelet function)
what is importance of uremia?
It inhibits platelet function. give them dialysis to remove organic acids! Uremia: kidney can't clear organic acids (guanidinosuccinnic acid)-->inhibits platelet function
most frequently acquired bleeding D/O due to abnl plasma coagulation are
liver Dz and DIC
how do you evaluate thrombocytopenia
do BM biopsy to access megakaryocytes
what is wrong in vWD
platelets are nl, but no vWF, so platelets can't adhere