Bleeding disorders

Question 1

describe thrombocytopenia

Answer 1

primary haemostatic disroder

Question 2

describe haemophillia

Answer 2

coagulation disorder

Question 3

what can cause bleeding defects

Answer 3

vascular defects
low platelet number
lowplatlet function
low coagulation

Question 4

what is thrombocytopenia

Answer 4

low platlet count

bewo 50x10^9/L

Question 5

causes of thrombocytopenia

Answer 5

failure of bone marrow production

short life span of RBC

Question 6

what is ITP

Answer 6

idiopathic thrombocytopenia purpura

sudden onset of immunologica destruction of platlet

Question 7

acute ITP

Answer 7

sudden onset in children after a trivial ilness
virus Ag bind to platlets and tag it as viral so its destroed
most will recover fine

Question 8

chronic ITP

Answer 8

gradual onset in adults with no previous infection
autoAb agaisnt the plaatelet membrane
platelts destroyed in spleen very quickly

Question 9

treatment for chronic ITP

Answer 9

reduce immune function with cortiocosteriods

splenectomy

Question 10

what is TTP

Answer 10

thrombotic thrombocytopenic purpra

formation of vWF multimers leading to platlet aggegation (thrombosis) and therefore thrombocytopenia

Question 11

acquired TTP

Answer 11

Ab block the protease breaking down vWF multimers

Question 12

familiaral TTp

Answer 12

protease to break dwn vWF multimers absent or defective

Question 13

DIC

Answer 13

disseminated intravascular cogulation
wide spread inapropriate deposition of fibrin leading to the consumption of coag factos and platlets leading to thrombocytopenia and bleedin

Question 14

causes of DIC

Answer 14

infections
malignancy
hyersensitivity reactions
tissue damage

Question 15

disorders of platlet function

Answer 15

inherited or acquired

Question 16

platlet adhesion defects: VDW

Answer 16

von willebrand disease
low factor 8
very common

Question 17

what are the three forms of VWD

Answer 17

quantitiative partial defieicnecy
functional abnormality
complete deficiency

Question 18

diagnosis of VWD

Answer 18

prolonged bleeding
normal platlet number
aPPT long
PT normal

Question 19

bernard-soulier syndrome

Answer 19

platler adhesion defects
isue with GP1b complex
rare
plaltelets cant bind to VWF, they dont stick down

Question 20

secretion defects

Answer 20

lead to low coagulaion and therefore bleeding

Question 21

grey platlet syndrome

Answer 21

platelts lack alpha granules and look grey and reduced agregation ability
secretion defect

Question 22

aggregation defects of platlets

Answer 22

glanzmann thrombasthenia
inability of platlets to bind fibrinogen and form a platlet plug
prolonged bleeding time as they fail to aggregate when needed

Question 23

platlet coagulation protein interaction defects

Answer 23

scott syndrome

inability of factors to bind to the platler surface resulting in lack of thrombin production

Question 24

examples of inherited platler function disorders

Answer 24

• adhesion defects
• agonist receptr defects
• signalling defects
• secreton defects
• aggregation defects
• coagulation protein interaction defects

Question 25

examples of acquired platelet functon disorders

Answer 25

• asprin
• chronic renal failure
• cardiopulmnary bypass
• haematological disases

Question 26

effect of asprin on platlets

Answer 26

irreversibly acetylates enzye which can lead to a risk of bleeding by affecting platle function

Question 27

how doe chronic renal failure impact platlet function

Answer 27

increase in bleeding due to-

• uraema, high nitrogen leading to platlet defects
• anaemia, less epo made in the kidney

Question 28

how does cardiopulmonary bypass affect platelets

Answer 28

platlet actvation and degranulation occurs in the extrcorporeal circuit outside of the body and can lead to excessve bleeding

Question 29

how can reduced coagulation occur

Answer 29

deficiency in a coagulation factor, either inherited or acquired
abnormailitties in fibrinogen

Question 30

haemophillia A

Answer 30

inherited coagultion deficiencies
lack of factor 8
x-linked, affects males

Question 31

treatment for haemophillia A

Answer 31

purified factor 8

Question 32

vWD

Answer 32

common diseasea

affects factor 8 leevls

Question 33

haemophillia B

Answer 33

deficiecy in factor 9
x linked, males
-require the factor to be replaced for treatment

Question 34

factor 11 deficeincy

Answer 34

recessive mutation, entirely with Ashkenazi jews

Question 35

factor 7 deficiency

Answer 35

affects extrincisc pathway

Question 36

factor 5 deficency

Answer 36

very rare

Question 37

acquire defienciesin coagulation examples

Answer 37

DIC
liver disase
vit K deficiency
acquired haemmophillia

Question 38

how does DIC lead to deficiencies in coagulation

Answer 38

inappropriate deposition of fibrin consumes the coagulation factors and platlets leading to defiencies in the factors

Question 39

how does liver disease lead to coagulation defiencies

Answer 39

clotting factors are made in the liver so in disease they arent made leading to bleeding isues

Question 40

how does a vit k defienciy lead to a clotting defienciy

Answer 40

clotting factors need vit K to be syntheised so if no Vit K, some clotting factors cant be made
this is what warfarin does

Question 41

what is acquired haemophillia

Answer 41

autoanitbodie against factor 8

leads to defiency and prolonged bleeding

Question 42

quanititative deficiencies in fibrinogen

Answer 42

apofibrinogenamiae
- complete lack, severe
hypofibrinogenaemia
- reduced amount, not so severe

Question 43

qualitative defiencies in fibrinogen

Answer 43

dysfinbrinogenaemia

- dysfunctional fibrinogen

Question 44

what are the main causes of bleeding defects

Answer 44

• vascular defects
• low platlet numbers
• reduced platlet funtion
• reduced coagulation function