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PATHOLOGY > Coagulation - bleeding disorders > Flashcards

Coagulation - bleeding disorders Flashcards

1
Q

What can genetic defects effect -> bleeding disorders

A

plt - most common either thrombocytopenia or because of abnormal function

blood vessel wall

clotting factor deficiencies - haemophilias

excess clot breakdown - fibrinolysis

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2
Q

which acquired defects can cause bleeding disorder

A

liver disease - clotting factor production
vit K deficiency
autoimmune disease -> plt destruction (thrombocytopenia)
trauma - damage to endothelium
anticoagulants/antiplatelets

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3
Q

genetic defects -> thrombosis

A

clotting factor inhibitor deficiencies

decreased fibrinolysis

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4
Q

disorders of haemostasis

A

vascular - scurvy, easy bruising

plt disorders - thrombocytopenia/abnormal function

coag disorder - factor def

mixed/consumption of coag factors and plts = disruption of coag system as whole -> DIC -> depletion of plt, clotting factors, fibrinogen -> life threatening condition, need to treat underlying cause

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5
Q

features in hx that suggest type of disorder

A

Local VS generalised

haematoma/joint bleed - haemophilia/severe form of other disorder

skin/mucosal petechiae/purpura - plts or VWD

wound/surgical bleeding - factor 13 def, general global clotting problems

immediate - primary haemoststic plug - plts, endothelium, vWF

delayed - coagulation factor 8 9 11 (depend on age, FHx, ethnicity)

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6
Q

why is bleeding with haemophilia delayed

A

no problem with primary plug - VWF, endothelium and plts

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7
Q

clinical features of bleeding disorders

A
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8
Q

what is this - and the type of bleeding disorder

A

petechiae
purpura

due to plt

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9
Q

what is this - and the type of bleeding disorder

A

haemarthrosis

coagulation

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10
Q

normal plt count

A

150-400

have to be much lower than 150 to get sx - so dont treat until less than 30 unless bleeding

Treat less than 30 - risk of intracerebral or mucosal haemorrhage -> fatal

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11
Q

examining plts under microscope

A

confirm true thrombocytopenia - can get pseudothrombocytopenia where plts clump - usually due to EDTA

see rare things:
grey-plt syndrome
inclusion bodies in white cells - Döhle body-like leukocyte inclusions in May-Hegglin syndrome
rare hereditory microthrombocytopenic conditions - low plts, large plts, little bleeding tendency

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12
Q

possible disorders of plts

A

thrombocytopenia
* decreased production
* decreased survival - autoimmune (ITP) - survival goes from 7-10days to a few hours
* increased utilisation - DIC

defective plt function
- acquired - drugs - aspirin, end-stage renal failure unless reg haemodialysis
- congenital - thrombasthenia - effect glycoproteins, granules, in plts - dx on platelet function analysis

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13
Q

mode of action of anti-plt drugs

A

a lot of plts on dual antiplt agent - aspirin and clopidogrel - esp if MI and stent to stop stent blocking

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14
Q

causes of thrombocytopenia

A

immune-mediated - related to immune dysregulation - plts recognosed as foreign and destroyed
* idiopathic
* drug-induced - quinine, rifampicin, vancomycin
* connective tissue disease - SLE, rheumatoid
* lymphoproliferative disease - CLL, leukaemias
* sarcoidosis

non-immune mediated
* DIC
* MAHA - blood cells haemolysed because of TTP

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15
Q

Pathophysiology of auto immune idiopathic thrombocytopenia

A

autoAb tag the plts
as go through reticuloendothelium system
destroyed by macrophages
-> thrombocytopenia

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16
Q

Differentiation of acute/chronic ITP

A

acute = children
chronic = adult

<20 is severe thrombocytopenia.
But children respond w/o Rx - dont get treated because risk of intracranial haemorrhage is rare

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17
Q

Rx of ITP

A

depends on bleeding sx and level of plt count

IVIG compete with the autoAb - block receptor fro teh reticuloendothelial system and allow plts to last longer

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18
Q

bleeding with ITP

A

Haematoma
petechiae - non-blanching
echymoses
sunconjunctival haemorrhage - superficial bleed

19
Q

Ix of ITP

A

Blood film
* low plts
* macrocyte - B12/folate def - associated with thrombocytopenia
* acute leukaemia - myeloid blasts, Auer rods - cause thrombocytopenia

20
Q

Inherited bleeding disorders - coagulation factor disorders

A

haemophilia A and B
vWB
other factor deficiencies (11, 10, 7, combined 8 and 5) - rare, present early in life, Rx with clotting factor

21
Q

acquired bleeding disorders - coagulation factor disorders

A

liver disease
vit K deficiency/warfarin OD
DIC

22
Q

Summarise haemophilia

A

congenital deficiency - factor 8 (A) or 9 (B)
bleeding = haematoma/joint
gene on X chr - carrier females, males suffer
long aPPT (intrinsic), normal PT

Rx- replace clotting factor - from plasma or recombinent factors

23
Q

genetic inheritence of haemophilia

A
24
Q

clinical manifestations of haemophilia

A

haemarthrosis (fixed joints) - most common
soft tissue haematomas (muscle) - muscle atrophy, shortened tendons
eccymoses
urinary tract bleeding
bleeding in CNS/Neck - life threatening
long bleeding after surgery/dentist

25
Q

difference between haemophilia A and B

A
26
Q

clinical features of vWD and inheritence

A

most common inherited bleeding disorder
most have mild disease only - heavy period/nose bleed
autosomal dominant
mucocutaneous bleeding

27
Q

Classification of vWD

A

type 1 - partial quantitative deficiency

type 2 - quantitative deficiency - function reduced

type 3 - total quantitative deficiency - very similar to haemophilia A because strong relationship between vWF and factor 8 - live together in circ, vWF protect factor 8 being destroyed in circ - if vWF not there = shorter life of 8

28
Q

sources of vit K

A

green veg
synthesised by intestinal flora

29
Q

what factors need vit K

A

2 7 9 10
protein C
protein S
protein Z

30
Q

Causes of vit K deficiency

A

malnutrition
biliary obstruction
malabsorption
antibiotic therapy

31
Q

Rx of vit k deficiency

A

vit K - oral, unless not going to absorb eg obstruction due to malignancy/stones - need IM but if impaired coag IM might = haematoma
fresh frozen plasma

32
Q

causes of DIC

A

sepsis
trauma - head injury/fat embolism
malignancy
obs complications - amniotic fluid embolism, abruption placentae
vascular disorder
reaction to toxin - snake venom, drugs
immunological disorders - severe allergic reaction, transplant rejection

33
Q

how serious is DIC

A

life threatening
could die in hours

34
Q

dx of DIC

A

lab-clinical dx
see lab changes
- deranged aPTT, PTT, low fibrinogen, low plt,
- factor 8 low
- all consumed by **activation of coag and fibrinolysis **

35
Q

paediatric case of DIC

A

meningococcal meningitis -> sepsis -> DIC

Rx - clotting factor, plts, Rx meningitis - stop the drive

36
Q

mechanism of DIC

A

deposition of fibrin -> kidney damage, brain damage, necrosis of extremities -> amputation

depletion of plts and coag factors -> bleeding

37
Q

Pathogenesis of DIC

A

release thromboplastic material into circulation ->
1. coagulation -> thrombin
2. fibrinolysis -> fibrin degredation product -> block circulation

38
Q

DIC blood film

A

red cell fragments - red blood cells get sliced by the fibrin meshwork
anaemia

39
Q

Rx of DIC

A

treat underlying disorder
anticoag with heparin
plts
FFP
maybe recombinant activated protein C

40
Q

how does liver disease -> coag problems

A

decreased synth of 2 7 9 10 11 and fibrinogen
dietary vit K def 0 inadequate intake/malabsorption
dysfibrinogemia - dysfunction of fibrinogen
enhanced fibrinolysis - decreaed alpha-2-antiplasmin (inhibitor for plasmin activator)
DIC
thrombocytopenia due to hypersplenism

41
Q

mx of haemostatic defects in liver disease

A

plasma and replace clotting factor when needed - as sx dictate

42
Q

managing high INR levels from warfarin

A

danger of bleeding is related to INR - higher = more risk of bleed

give Vit K or FFP or prothrombin complex concentrate containing 2 7 9 10 (because warfarin is vit K antagonist)

43
Q

DOACs

A

Dont have an antidote

PATHOLOGY (94 decks)

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