Path-Heme-Bleeding D/o

Front 1

Tx for vWF Dz

Back 1

demospression (DDAVP)

Front 2

Tx for Factor 8 deficiency (Hemophilia A)

Back 2

desmopression (DDAVP)

Front 3

Tx for deficiency of all other coagulation factors besides Factor 8 and 9

Back 3

FFP;
except hypofibrinogenemias--Tx with cryoprecipitate

Front 4

what is liver's role in hemostasis

Back 4

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)

Front 5

hemostatic effects of liver Dz

Back 5

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

Front 6

Vit K is what

Back 6

Vit K=fat soluble quinones derived from plants (K1) or microorganisms (K2)

Front 7

what is the Vit K cycle

Back 7

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

Front 8

Vit K deficiency causes what problems

Back 8

1. HDN
2. Malabsorption Syndromes (caused by biliary obstruction)
3. Intestinal sterilization (loss of K2 from enteric bacteria)

Front 9

what meds can cause bleeding

Back 9

(anticoagulants)
1. Warfarin (oral)
2. Heparin

Front 10

what causes thrombocytopenia

Back 10

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)

Front 11

Tx Thrombocytopenia

Back 11

1. treat underlying D/O
2. platelet transfusions
3. splenectomy

Front 12

Sx of thrombocytosis

Back 12

thrombosis (platelet>1 million) OR bleeding

Front 13

Sx of von Willebrand Dz

Back 13

bleeding in skin, mucus membranes, GI, and urinary tract

Front 14

what technique used to classify VW Dz

Back 14

multimeric analysis

Front 15

classification of VW Dz

Back 15

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

Front 16

is the mcs in Type 2 vWD normal or abnl?

Back 16

abnl

Front 17

pathogenesis of vWD

Back 17

a. defect in vWF subunit structure
b. defective platelet-vWF interaxn

Front 18

how Tx vWD

Back 18

vWF replacement

Front 19

Bernard-soulier syndrome

Back 19

1. platelet membrane defect: GP1b-9 complex absent
2. decreased aggregation in response to vWF (+ristocetin)
3. nl aggregation to ADP, collagen, EPI, thrombin

Front 20

thrombasthenia

Back 20

1. platelet membrane defect: GP2,3 absent
2. decreased agg in response to ADP, collagen, EPI, thrombin
3. nl agg to vWF (+ristocetin)

Front 21

what are the inherited Qualittive Platelet D/O

Back 21

1. vWD
2. Bernard-Soulier
3. Thrombasthenia
4. Storage Pool D/O

Front 22

Storage Pool D/O involves what?

Back 22

dec platelet agg b/c:
1. defective SERO uptake into platelets
2. defective SERO release from platelets

Front 23

acquired qualitive platelet D/O?

Back 23

**Uremia: poor platelet fxn b.c inhibited by retained organic acids (guanidinosuccinic acid)
-drug (aspirin)
-liver dz
-myeloprolif d/o
-paraproeinemias

Front 24

Pt w/renal failure and GI bleed. Platelet transfusion doesn't work. What do you do?

Back 24

give them dialysis to remove organic acids! (Uremia: kidney can't clear organic acids (guanidinosuccinnic acid)-->inhibits platelet function)

Front 25

what is importance of uremia?

Back 25

It inhibits platelet function. give them dialysis to remove organic acids! Uremia: kidney can't clear organic acids (guanidinosuccinnic acid)-->inhibits platelet function

Front 26

most frequently acquired bleeding D/O due to abnl plasma coagulation are

Back 26

liver Dz and DIC

Front 27

how do you evaluate thrombocytopenia

Back 27

do BM biopsy to access megakaryocytes

Front 28

what is wrong in vWD

Back 28

platelets are nl, but no vWF, so platelets can't adhere