Lec 7 - Exam 1 Heme

Front 1

Type of disorders cause excess bleeding.

Back 1

DIC, Vitamin K deficency, massive trasfution, primary fibrinogenolysis, inhibitors of fibrinolysis, deficiencies of coagulation factors such as heriditory deficiencies, acquired deficiencies, and acquired inhibitors. .

Front 2

types of coagulation factor deficiencies that cause excess bleeding

Back 2

heriditory deficiencies, acquired deficiencies, and acquired inhibitors.

Front 3

Saverity of heriditory deficiencies of coagulation factors

Back 3

varies on diseases, fairly rare, varied inheritence patterns.

Front 4

Saverity of heriditory deficiencies of coagulation factors

Back 4

usuallt excess bleeding, sometime thromboses instead.

Front 5

treatment for heriditory deficiencies of coagulation factors

Back 5

only needed when activity bleeding, or give prophylaxis before surgery.

Front 6

Fibrinogen deficiency treatment

Back 6

with cryoprecipitate that is a HMW proteins that preciplitate our in the cold such as vWF, VIII, XIII, and fibrinogen

Front 7

cryoprecipitate drug components

Back 7

HMW proteins that preciplitate our in the cold such as vWF, VIII, XIII, and fibrinogen. Used to treat fibrinogen deficency.

Front 8

treatment for prothrombin deficiency

Back 8

with prothrombin complex concentrate or FFP -fresh frozen plasma.

Front 9

Factor V ( labile factor deficiency) treatment

Back 9

treat with cryo-free plasma or FFP if activity bleeding.

Front 10

Factor VII ( stable factor deficiency) treatment

Back 10

treat with plasma or prothrombin complex concentration

Front 11

factor defect in hemophilla A

Back 11

facter VIII C. facter VIII deficency is sex linked. Only in males.

Front 12

Most common inherated disorder of bleeding

Back 12

vonWillebrand Disease

Front 13

deficiency in vonWillebrand facter part of the

Back 13

part of the facter VIII complex.

Front 14

diagnosis for vonWillebrand disease

Back 14

based on two assays. vWF factor functional activity and vWF antigenic properties of the factor.

Front 15

why testing for vonWillebrand disease is tricky

Back 15

vWF levels vary for many reasons. Increased in stress, different blood group has different level of vWF, depent on sample processing including cfg time if frozen plasma.

Front 16

vonWillebrand disease classification

Back 16

types 1,2,3. within type 2 ave 4 subunits.

Front 17

inheritence on vonWillebrand disease

Back 17

autosomal dominant

Front 18

hemostatis that affeted with vonWillebrand disease

Back 18

borh primary and secoundary. In primary its cuntion is to platlet adhesion , and secoundary to stabilisation of facter VIII C.

Front 19

list blod groups based on level of vWF

Back 19

AB > B > A > O

Front 20

Facter VIII C deficency charectorestic

Back 20

Hemophilia A, X linked, normal vWF , inherited as mild moderate and severe.

Front 21

mild moderate and severe lervels of Facter VIII C deficiency

Back 21

mild - 6- 30 % facter VIII levels, moderate 2 - 5% facter VIII levels, severe is < 1 % of the VIII levels.

Front 22

facter VIII C mild treatment

Back 22

sometime wih DDAVP - desmopressin.

Front 23

facter VIII C moderate or severe treatment

Back 23

cryoprecipitate of needed to stop bleeding. Give facter VIII concentrates routinely.

Front 24

Facter IX deficiency condition

Back 24

hemophilia B, X linked

Front 25

Facter IX deficiency treatment

Back 25

give facter IX concentrates or concentrated of prothrombin complex incluse 2, 7, 9, and 10

Front 26

treatment for facter X deficiency

Back 26

with FFP, cryp-free plasma or prothrombin group concentrates.

Front 27

if you have prothrombin time normal, prolonged aptt, and pt. positible dificency

Back 27

facter X deficiency,

Front 28

facter XI deficiency condition and treatment

Back 28

hemophilia C, treat with FFP.

Front 29

charectorestics of facter XII deficiency ( Hangeman Factor)

Back 29

not a bleeding problem, more likely thrombosis, usually discovered by accident, NO therapy required.

Front 30

Facter XIII deficiency treatment

Back 30

with cryoprecipitate.

Front 31

charectorestics of pre-kellekrein deficiency

Back 31

NO bleeding sysmtoms, more likely thromboses like facter XII def. No treatment necessary. Problem in vitro testing

Front 32

charectorestics of HMWK deficiency

Back 32

no treatment necessary, problem in lab testing like . pre-kellekrein deficiency

Front 33

chrectorestics of acquired deficiencies of coagulation factors

Back 33

more likely more than one factor ingerited disorde with varieous reasons

Front 34

reasons for acquired deficiencies of coagulation factors

Back 34

Facter synthesis, Diet, Malabsorbtion, Drug reaction, Complication with anticoagulant therapy.

Front 35

charectorestics of synthesis problem in acquired coagulation deficiencied

Back 35

problem in liver disease. All the factors expect factor VIII made in liver.

Front 36

treatment in synthesis problem in acquired coaguation problem

Back 36

plasma trasfution - FFP trasfution.

Front 37

one facter that is not made in liver

Back 37

facter VIII

Front 38

charectorestics of diet and absortion problem in acquired coagulation deficiencies

Back 38

viramin K deficiency, Vit K is required to prodice factor 2, 7, 9, and 10.

Front 39

conditions that can have vitamin K deficiency

Back 39

long term antibiotic therapy, malabsobtion disease, newborns.

Front 40

How is vtamin K made and absorbed

Back 40

made by normal bowl flora with bacteria and absorbed through intestine

Front 41

charectorestics of drug reaction problem in acquired coagulation deficiencied

Back 41

when taking anticoagulants such as coumadin and warfarin. They are viramin K antagonists. Can lead to excessive bleeding.

Front 42

Acquired pathologic inhibitors are associated with

Back 42

another underlying disease or de-novo. All inhibitors are antibodies such as IgG or IgM.

Front 43

Acquired pathologic inhibitors are directed towards

Back 43

single factor and antiphospholipids - lupus anticoagulants

Front 44

Most common Single factor in Acquired pathologic inhibitors

Back 44

anti facter VIII or anti factor IX.

Front 45

Lupus anticoagulation in Acquired pathologic inhibitor associated with

Back 45

SLE, cancers, infections, drug reaction, other autoimmune diseases.

Front 46

massive trasfution probem

Back 46

cause facter dulution and stored blood may contain depleted quantities of factors, especially the labile ones.

Front 47

Charectorestics of DIC

Back 47

massive clotting and consumption of platlets and factors cause massive bleeding.

Front 48

Condition in primary fibrinolysis

Back 48

fribrigenoGENolysis.

Front 49

Primary fibrinolysis charectorestics

Back 49

when tissue damage in condition such as cancers , plasminogen activators from damaged tissue is released that casue formation of plasmin. Plasmin cause fibrinolysis, and fibrinogenolysis which can lead to FSP's ( anticoagulants). Patient BLEED

Front 50

What is thrombophilia

Back 50

Thrombosis, condition resulting in excess cloting

Front 51

Inherited thromboses are deficiencies in

Back 51

Inhibitors.

Front 52

type of deficiencies of inhibitors that lead to thromboses

Back 52

Antithrombin, Protein C or Proein S, Hyperhomocysteinemia, Lupus, Miscellaneous.

Front 53

Condition in acquired thromboses

Back 53

DIC

Front 54

Charectorestics of antithrombin deficiency

Back 54

thromboses, reducedregulation og thrombin cause excess cloting.

Front 55

how to test for antithrombin deficiency

Back 55

thrombin ingibition testing of facter Xa

Front 56

how to treat antithrombin deficiency

Back 56

treat with hepran, and warfarin later.

Front 57

Inherited thromboses deficiencies that treated with warfarin

Back 57

Protein C and Pretin S deficency .

Front 58

Protein C deficiency analysed by

Back 58

Chromogenic substrate testing, and Clot based testing- that protein C prolonges aPTT by inactivation of V and VIII

Front 59

Clot based testing result on protein C deficiency

Back 59

that protein C prolonges aPTT by inactivation of V and VIII

Front 60

Symtoms of protein C , Protein S and antithrombin deficiency

Back 60

PE and thrombophlebitis.

Front 61

methrods to detect protein C deficiency

Back 61

ELISA and Immunoassay for total protein S. Clot based test for protein C , protein S and activated factor V.

Front 62

pathological causes of Hyperhomocysteinemia

Back 62

cause due to b12/foliate deficiency. Mutation in enzymes for metabolism of homocysteine to methionine.

Front 63

Hyperhomocysteinemia resuls in increasd risk of

Back 63

increase risk of atherosclerosis, and thromboses.

Front 64

laboratory assays for Hyperhomocysteinemia

Back 64

vie various chromatography techniques

Front 65

Activated protein C resistance ( facter V-leiden) chrectorestics

Back 65

mutant factor V is resistant to inhibition by protein C. Clot based scrren and molcular methors ro conform in ref labs.

Front 66

risk of women who are on OCP ( oral contrcept) to have Activated protein C resistance to have a pathological clots

Back 66

30 times than normal

Front 67

risk of women who are on OCP ( oral contrcept) to do not have Activated protein C resistance to have a pathological clots

Back 67

2 times than people without on OCP- oral contracept.

Front 68

Facter V leiden test methodology

Back 68

by PCR of factor V gene - MnII amplicon is used.

Front 69

mulation that caused in facter V leiden

Back 69

replacent of arginine to glutamine in the factor V protein at position 506.

Front 70

reason for increased prothrombin level

Back 70

gene mutation. G to A base mutation. Cause thromboses.