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Introduction to Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

symptoms of bleeding disorder

A

epistaxis (bleeding from nose)
bruising
purpura
petechiae
menorrhagia
joint bleeds
muscle bleeds
chronic anaemia

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2
Q

primary haemostatic defects symptoms

A

small bruises
epistaxis
prolonged bleeding from cuts and mucous membranes
bleeding after dental extraction or surgery

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3
Q

secondary haemostatic defects symptoms

A

large bruises at unusual sites
haematomas
joint and soft tissue bleeds
haematuria
prolonged bleeding

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4
Q

examples of acquired bleeding disorders

A

vitamin K deficiency
liver disease
massive haemorrhage
disseminated intravascular coagulation (DIC)
autoantibodies/inhibitors

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5
Q

examples of inherited bleeding disorders

A

haemophilia
VWD
bernard-soulier syndrome
glanzmann’s thrombasthenia

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6
Q

what is vitamin k

A

a fat soluble vitamin

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7
Q

where is vit k found

A

green leafy veg and liver

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8
Q

causes of vit k deficiency

A

malabsorption of fat soluble vitamins
liver disease
oral anticoagulants (e.g. warfarin)
antibiotics that destroy gut bacteria
newborn infants have low vit k - given vit k injections

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9
Q

what is the function of vit k

A

serves as an essential cofactor for a carboxylase that catalyses carboxylation of glutamic residues on vit k dependent proteins

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10
Q

what are examples of vitamin K dependent proteins

A

coagulation proteins - FII (prothrombin), FVII, FIX and FX
anticoagulation proteins - protein C, protein C

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11
Q

what is required for vit k to be functionally active

A

post-translational modification

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12
Q

what is the vit k cycle

A

gamma carboxylation of glutamic acid allows for strong chelation of calcium
induces a shape change in vit k dependent proteins
positively charged calcium allows proteins to bind to negatively charged phospholipids

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13
Q

what are the vitamin K dependent factors in the coagulation cascade

A

FII (prothrombin)
FVII
FIX
FX
Protein C
Protein S
present but functionally inactive

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14
Q

what happens to bleeding in vit k deficiency

A

bleeding tendency and prolonged clotting times (PT and APTT)

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15
Q

what can liver disease be caused by?

A

viruses, alcohol abuse, obesity or inherited

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16
Q

what clotting factors does the liver produce?

A

FI, II, V, VII, IX, X and some VIII

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17
Q

what is the order of reduction in liver disease

A

FVII -> FII -> FX -> FI -> FV

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18
Q

what happens in liver disease

A

decreased clearance of activated factors and inhibitors. thrombopoietin produced in liver and kidney so decreased platelet count. FI and FV persist even in severe liver disease

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19
Q

what is a massive haemorrhage

A

acute blood loss including the loss of:
rbcs
platelets
coagulation factors cytokines and chemokiines

clinical shock or blood loss of >50% of circulating volume within 3hours or blood loss >150ml/min

consumptive coagulation

combo of blood components required to restore effective haemostasis

transfusion may be risky but bleeding to death is always fatal

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20
Q

who are platelet transfusions indicated for

A

the treatment or prevention of bleeding in patients with thrombocytopenia or platelet dysfunction

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21
Q

platelet transfusion info

A

store 20-24 degrees c agitated shelf life 5-7 days
1 ATD platelets contains ~ >240x10^9/L
give ABO and RhD matches where possible

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22
Q

what does cryoprecipitate contain

A

FVIII, VWF, Fibrinogen, FXIII and fibronectin

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23
Q

what is octaplas a source of

A

clotting factors (decreased protein S)

24
Q

what is disseminated intravascular coagulation (DIC)

A

consumptive coagulopathy

widespread inappropriate intravascular deposition of fibrin with consumption of coagulation factors and platelets

can result in both thrombocytopenia, bleeding and multiple organ damage

widespread activation of the clotting cascade that results in the formation of blood clots in the small blood vessels throughout the body

25
Q

what are the causes of DIC

A

infections / sepsis
malignancies
obstetric complications
widespread tissue damage

26
Q

primary events in DIC

A

underlying disorder
systemic activation of coagulation
widespread intravascular fibrin deposition - thrombosis / consumption of platelets and clotting factors -bleeding

27
Q

what does thrombin catalyse?

A

the conversion of fibrinogen to fibrin

28
Q

what does fibrinolysis generate?

A

D-dimers from cross-linked fibrin

29
Q

treatment of DIC

A

treatment of the underlying disease:
- more likely to die from this than thrombosis or bleeding
- Abx, surgery, chemo, embolization
- disease specific therapy (e.g. APL- all trans-retinoic acid (atra))

replacement therapy:
- support with blood products to treat bleeding
- platelets
- FFP
- Cryoprecipitate

outdated therapies used to treat thrombosis (chronic DIC) include:
- heparin - was given as an anti-coagulant (but also used to reduce clotting factor consumption and secondary fibrinolysis)
-recombinant human activated protein C (previously recommended for patients with severe sepsis and DIC)

30
Q

autoantibodies/inhibitors

A

development of antibodies or inhibitors which bind to and neutralise clotting proteins

antibodies may be directed against isolated clotting factors such as VIII or IX inhibitors

can appear patients with no hereditary disorder of coagulation

can be associated with some autoimmune conditions

antiphospholipid antibodies are known to develop against multiple coagulation proteins

31
Q

how is haemophilia A treated

A

with recombinant FVIII, ~14% of these patients will develop inhibitory antibodies compared to ~2% haemophilia B patients

32
Q

what is congenital haemophilia

A

X-linked congenital bleeding disorder caused by genetic mutations of clotting factor genes

33
Q

prevalence of haemophilia A and B

A

1 in 10,000 males
1 in 50,000 males

34
Q

what is haemophilia A

A

deficiency of FVIII factor 8

35
Q

what is haemophilia B

A

deficiency of factor IX factor 9

36
Q

what is haemophilia C

A

deficiency of FXI (11)

37
Q

haemophilia genetics

A

F8 gene located on telomeric end of X chromosome (Xq28) - 2332 aa. >2000 mutations detected in F8 gene.

F9 gene located on telomeric end of X chromosome (Xq27)- 415 amino acids. 1095 mutations found in F9 gene.

38
Q

what % of carrier females of haemophilia are at risk of bleeding

A

10%

39
Q

female haemophiliacs conditions

A

lyonisation
turner syndrome (XO)
true haemophiliac
normandy VWD

40
Q

severity of clotting factor deficiencies

A

severe <1% of normal
moderate 1-5% of normal
mild 5-<40% of normal

normal range is 5-150% factor activity in blood

41
Q

treatment of haemophilia - prophylaxis:

A

plasma derived clotting factors
recombinant clotting factors
DDAVP (Desmopressin)
why?
cheaper than on demand treatment
decreased transfusion transmitted infection e.g. HIV/HEPC
decreased TACO transfusion associated circulatory overload
but?
increased inhibitor development
portacath access = increased infection and thrombosis risk

42
Q

von willebrand disease

A

autosomal bleeding disorder of varying clinical severity
most common type of inherited bleeding disorder present in ~1% of population
classically presents as a mild-moderate bleeding disorder

43
Q

symptoms of vwd

A

epistaxis
bruising
excessive but rarely life threatening bleeding
excessive minor wound bleeding
heavy menstruation

44
Q

what are the 3 distinct types of vwd:

A

type 1: partial quantitative deficiency of vWF
type II: qualitative dysfunction of vWF
type III: total vWF defciiency

45
Q

what does vwf do

A

vWF protects FVIII from proteolytic degradation, decreased vwf=decreased fviii=increases bleeding

46
Q

what are the 2 main treatment options for vwd:

A

desmopressin DDAVP for type 1 vwd
vwf/fviii concentrates

47
Q

what can antifibrinolytic drugs (tranexamic acid) be used for?

A

to treat mild mucocutaneous bleeding

48
Q

what can topical agents (e.g. fibrin sealents) be used for

A

dental surgery and for surface wound bleeding

49
Q

who may platelet transfusions be useful for?

A

some patients with vwd e.g. type 3

50
Q

what can be used to help restore VWF and FVIII levels?

A

cryoprecipitate and FFP

51
Q

what can platelet disorders cause?

A

abnormal/excessive bleeding

52
Q

quantitative thrombocytopenia

A

increased destruction or decreased production

53
Q

what else can cause platelet disorders

A

defective platelet function

54
Q

what ate platelet disorders characterised by

A

spontaneous skin purpura, mucosal haemorrhage and prolonged bleeding after traum

55
Q

what is likely to be more severe if platelet production failure is the root cause

A

bleeding

56
Q

what can cause thrombocytopenia (decreased platelets)

A

genuine ALD, ITP, ALL
Platelet clumps
platelet satellitism
clotted (fibrin strands)

last 3 are psuedothrombocytopenia

Introduction to Haematology (10 decks)

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