bleeding disorders Flashcards

1
Q

points to cover in bleeding hx

A

has the patient actually got a bleeding disorder

how severe is the disorder - what does it take to make the patient bleed

pattern of bleeding

congenital/acquired

mode of inheritance

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2
Q

hx of bleeding

A

bruising
epistaxis
post surgical bleeding - dental surgery, circumcision, tonsillectomy, appendicectomy
menorrhagia (good -ve predictive factor for vW disease)
post-partum haemorrgage
post-trauma

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3
Q

what score can be done for bleeding

A

BAT score

gives you a quantitative score for assessing bleeding symptoms

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4
Q

severity of bleeding in hx

A

how appropriate is the bleeding for the situation

level of trauma - is the bleeding expected or far more than normal

any unprovoked bleeding - indicative of severe coagulopathic abnormality

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5
Q

platelet type pattern of bleeding

A
mucosal 
epistaxis - doesn't stop or stops and quickly restarts again
purpura
menorrhagia
GI

post-surgical/post-interventional bleeding

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6
Q

coagulation factor pattern of bleeding

A
articular - esp hinge joints e.g. knee, ankle, elbow
muscle haematoma (spontaneous of after minor injury)
CNS
post intervention/surgery bleeding
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7
Q

classic feature of petechiae

A

don’t blanch when pressed

red lesions that would blanch would have vessel involvement e.g. telangiectasia, arterioles, spider veins etc

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8
Q

how does haemophilic arthropathy occur

A

bleeding into the joint

iron released from red cells is taken up by macrophages

inflammatory response

boggy synovitis as a result

substances released prevent the repair of normal cartilage in the joint

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9
Q

how to determine is bleeding is congenital or acquired

A

previous episodes - also prev episodes of trauma, when were they

age at first event

prev surgical challenges

associated hx - FHx of bleeding disorder

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10
Q

hx of hereditary bleeding disorders

A

family members w/ similar hx

sex - determines nature of inheritance

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11
Q

haemophilia A and B

  • inheritance
  • phenotypes
  • prevalence
A

X linked

identical phenotypes

1/10 000 (A) and 1/60 000 (B)

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12
Q

what determines severity of bleeding in haemophilia A and B

A

severity of bleeding depends on the residual coagulation factor activity

<1% severe, residual level of factor VIII and IX 1-5% moderate, 5-30% mild

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13
Q

clinical features of haemophila

A

haemarthrosis

muscle haematoma

CNS bleeding

retroperitoneal bleeding

post-surgical bleeding

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14
Q

clinical complications of haemophilia

A

synovitis

chronic haemophilic arthropathy

neurovascular compression (compartment syndromes)

other sequelae of bleeding (haemorhaggic stroke)

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15
Q

diagnosis of haemophilia

A

clinical hx
coagulation tests: APTT (activated partial thromboplastin time), PT
- prolonged APTT (coagulation factor deficiencies - reduced factor VIII or IX)
normal PT
reduced factor VIII or IX

genetic analysis - specific abnormality within the family causing the condition, used for forward planning

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16
Q

haemophilia treatment of bleeding diathesis

A

coagulation factor replacement - VIII/IX
- now almost entirely recombinant products

DDAVP - desmopression (can allow you to avoid recombinant/plasma products)
TXA - reduces the rate of clot removal
emicizumab - monoclonal ab which binds to F X and IX - provides the function of F VIII

emphasis on prophylaxis in severe haemophilia
gene therapy - produce normal F VIII/IX for the pt

17
Q

treatments for symptoms of haemophilia

A
splints
physiotherapy 
analgesia
synovectomy 
joint replacement 
  • management of haemophilic arthropathy and muscular haematoma
18
Q

complications of haemophilia treatment

A

viral infection from transfusion of blood products - IIIV, HBV, HCV, others - vCJD

inhibitors - develop anti F VIII ab (25% of pts), rare in F IX

DDAVP - MI, TIA, hyponatraemia (babies)

19
Q

von willebrand disease

  • prevalence
  • severity
  • inheritance
A

1/200

variable severity

autosomal

platelet type bleeding (mucosal)

quantitative and qualitative abnormalities of vWF

20
Q

vWF deficiencies in vWD

A

type 1 quantitative deficiency (reduced quantity but maintains all function, reduced production/increased clearance)

type 2 (A,B,M,N) qualitative deficiency determined by the site of mutation in relation to vWF function

type 3 severe (complete) deficiency

21
Q

treatment of vWD

A

vWF concentrate or DDAVP - elevate vWF level
TXA (tranexamic acid) - for heavy menses and after surgical procedures
topical applications - e.g. dental
OCP etc - heavy menorrhagia

22
Q

when can DDAVP be used in vWD

A

type 1 disease

can’t use for very young children and individuals w/ significant CV risk - use vWF instead

23
Q

vWF for vWD

A

use recombinant concentrates where possible (no dependency on blood donor)

avoid plasma derived concentrates wherever you can

24
Q

acquired bleeding disorders

A
thrombocytopenia
liver failure 
renal failure
DIC
drugs: warfarin, heparin, aspirin, clopidogrel, rivaroxaban, apixaban, dabigatran, bivalirudin etc
25
Q

what causes thrombocytopenia

A

decreased production: marrow failure, aplasia, infiltration

increased consumption: immune ITC, non immune DIC, hypersplenism

26
Q

clinical features of thrombocytopenia

A

petechia

ecchymosis - dark purple bruise

mucosal bleeding

rare - CNS bleeding

27
Q

what is ITP

A

Immune thrombocytopenic purpura

adults and children

associations: infection (esp EBV, HIV - seroconversion illness), collagenosis (RA, SLE, connective tissue disease), lymphoma, drug induced

blood isolated thrombocytopenia

marrow

28
Q

drugs associated w/ ITP

A

vancomycin

teicoplanin

quinine

29
Q

treatment of ITP

A

steroids - high dose

IV IgG - reduces uptake of sensitised platelets by spleen

splenectomy

thrombopoietin agonists (eltromobopag, romiplostim, fostamatinib)

30
Q

bleeding in liver failure - why

A

reduced factor I, II, V, VII, VIII, IX, X, XI
reduced fibrinogen
prolonged PT, APTT

cholestasis - reduced vit K absorption, factor deficiency

31
Q

bleeding in liver failure

A

skin

GI - haemorrhoids, varices

increased risk of bleeding and thrombosis

32
Q

treatment of bleeding in liver failure

A

factor II, VII, IX, X
replacement FFP
vit K

33
Q

why is there bleeding and thrombosis in liver disease

A

reduced procoagulant and anticoagulant function

little insult in either direct could reduce either one and they can easily become unbalanced

results in bleeding/thrombosis or both

34
Q

haemorrhagic disease of the newborn

A

immature coagulation systems

vit K deficient diet (esp breast)

fatal and incapacitating haemorrhage

35
Q

how to prevent haemorrhagic disease of the newborn

A

completely preventable by administration of vit K at birth (IM/SC)