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Haematology > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

1
Q

what is clotting?

A

a system to repair defects in vessel wall
1’ haemostasis: platelet plug
2’ haemostasis: formation of a fibrin clot
(fibrin degradation- d-dimer)

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2
Q

what is needed for normal haemostasis?

A 
procoagulant proteins
anticoagulant proteins
fibrinolytic system
intact vascular endothelium
platelets (APPT)

*thrombin activates most

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3
Q

content of the platelets

A

no nucleus
procoagulants and anticoagulant
rich in other clotting factors such as VIII and VWF and V. Ca2+, ATP.

central role- initial closure in the vessel wall.

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4
Q

APTT

A

intrinsic pathway

amplifier of clotting. initiated by PT > amplified by APTT

???happens on the surface of the platelets because it needs phospholipid. (anti phospholipid antibodies?)
clotting test= in lab you ad the phospholipid
in vivo the anti phospholipid are procogaulnt?!?

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5
Q

coagulation factors

A

synthesised by the liver
sequence of reactions when activated
ends in the deposition of fibrin
network of reaction integrally involved with platelets

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6
Q

APTT

A

amplifier of clotting
initiated by PT > amplified by APTT

happens on the surface of the platelets because it needs phospholipid. (antiphospholipid antibodies?)

clotting test= in lab you ad the phospholipid

in vivo the antiphospholipid are procoagulant?!?

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7
Q

lab tests

A

CBC- plt
BT (CT)
PT
PTT

platelet study:
morphology, function, antibody

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8
Q

important coagulation factors (clinically)

A 
factor VIII (haemophilia A)
factor IV (haemophilia B)
VWF
factor VI ('haemophilia C' deficiency-increase risk of bleeding disorder in Jews?)
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9
Q

blood vessel injury

A

constriction (local nitric oxide, the release of epinephrine) reduces the calibre of the blood vessel (reduces surface area but increases the velocity)

platelet aggregation
coagulation cascade

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10
Q

thrombin

A

activated platelet
fibrinogen to fibrin
activates 9, 11, 5
activates matura anticoagulants.

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11
Q

factor V liden

A

less sensitive to the deactivation of protein C

generate thrombin for a longer time

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12
Q

site of bleeding

A

cuteness / mucus membranes (blood vessels capillaries cannot constrict as they have no smooth muscle so usually bleeding is just stopped by platelets)= platelet type bleeding disorder

deep in tissue / joints= dominated by coagulation protein disorders.= haemophilia

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13
Q

duration of bleeding

A

prolonged bleeding time= platelets

how long has the bleeding disorder been for?

  • chronic (congenital)
  • acute (late-onset) acquired haemophilia / VWF acquired

precipitating cause
delayed bleeding
surgery/procedures

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14
Q

site of bleeding

A

cuteness / mucus membranes: bruising, purpura, epistaxis, superficial cuts menorrhaga. (blood vessels capillaries cannot constrict as they have no smooth muscle so usually bleeding is just stopped by platelets)= platelet type bleeding disorder / VWF

deep in tissue / joints= dominated by coagulation protein disorders.= haemophilia

*ask about menorrhea.

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15
Q

systemic illnessess

A 
renal disease
liver disease
sepsis
DIC
paraproteins
drugs: aspirin, anti platelets, chemotherapy, DOACs
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16
Q

investigations for bleeding disorder

A

FBC
blood film examination
renal and liver function
(3) coagulation screen= APPT, PTT, fibrinogen
platelet function test including bleeding time.

fibrinogen is needed in active bleeding (polytrauma / major obstetric haemorrhage)
rate-limiting step - if consumed - ran out - bleeding (cannot form a stable clot) *measure using rotam

17
Q

VWF deficiency

A

most common inherited cause of abnormal bleeding (haemophilia)

different underyling causes of disease- autosomal dominant.

deficiency, absence or malfunction of glycoprotein- von willerbrand factor.

three types (1-3)

essential for platelet / subendothelial interaction
carrier for factor VIII (shields from being broken down)
quantitative/qualitative defects
PT is normal
APPT is prolonged (depends on VIII level)

  • VWF antigen
  • VWF activity

augment with desmopressin to release VWF from the cells- boost

18
Q

VWF deficiency

A

unusal easy, prolonged or heavy bleeding

Bleeding gums with brushing
Nose bleeds (epistaxis)
Heavy menstrual bleeding (menorrhagia)
Heavy bleeding during surgical operations
fhx of heavy bleeding

essential for platelet / subendothelial interaction
carrier for factor VIII (shields from being broken down)
quantitative/qualitative defects
PT is normal
APPT is prolonged (depends on VIII level)

  • VWF antigen
  • VWF activity

augment with desmopressin to release VWF from the cells- boost (DDAPD)

19
Q

haemophilia

A

deep in tissue- swollen knees (haemoarthrosis)

chronic haemophilia arthropathy (waisting of the muscles)

tx: name ? bite antibody bringing factor 5 and 9 = does the job of factor X.
subcutaneous injection

20
Q

haemophilia A or B

A

inherited severe bleeding disorders.

Haemophilia A is caused by a deficiency in factor VIII.

Haemophilia B (also known as Christmas disease) is caused by a deficiency in factor IX.

x linked recessive

factor VIII / IX
joint muscle, brain, haematuria, delayed post-op bleeding

prolonged APPT / normal PT

incidental finding on heel prick test

treatment:
minor- desmopressin
tranexamic acid
major- recombinant factor 8

21
Q

immune thrombocytponeia

A

purpuric spots

platelet count 15
blood film confirms true low count
no platelet clumps
normal Hb and WBC
ITP occurs in otherwise well

quality of platelets is good but not enough of them. less risk of life-threatening bleeding

  • lymphomas (CLL)
  • autoimmunity

treatment: suppress the immune system with steroids/ 2’/ splenectomy/ give dugs to increase platlets counts (thrombomimetics)

22
Q

myelodysplasia

A

knackered bone marrow
low platelets are part of bone marrow dysfunction (ineffective haemopooies)

blood film- diagnostic with abnormal platelets, RBC, wBC

quantitative and qualitative defect.

23
Q

hereditary haemorrhagic telangiectasia

A

predominantly around mouth and alimentary tract

can be in lungs and brains

24
Q

hereditary haemorrhagic telangiectasia

A

autosomal dominant
vascular malformation in skin, nasal membranes, gut, lung, brain, liver
more numerous in life
small feeding vessels

predominantly around mouth and alimentary tract

can be in lungs and brains

press- blood goes way, release- sudden blanching through the spiders.

need iron supplements because they bleed so much through their gut.

25
Q

Disseminated intravascular coagulopathy (DIC)

A

everything has been activated and consumed

sepsis, malignancy, severe ill health
consumption of clotting factors (consumptive coagulopathy)

APPT and PPT prolonged
fibrin increased
D dimer
platelet low

treat: FFP and platelets and the underlying cause.

26
Q

senile purpura

A

atrophy of supporting tissues of cutaneous blood vessels

dorsal aspect of forearm and hand worse if on steroids (thin)

27
Q

haemophilia pattern of inheritance

A

X linked recessive.

In order to have the condition all of the X chromosomes need to have the abnormal gene.

Men only require one abnormal copy as they only have one X chromosome.

Women require abnormal copies on both their X chromosomes, and if only one copy is affected they are a carrier of the condition.

Therefore haemophilia A and B almost exclusively affect males. For a female to be affected they would require an affected father and a mother that is either a carrier or also affected.

28
Q

haemophilia signs and symptoms

A

severe bleeding disorders. excessive bleeding from minor trauma
risk of spontaenous haemorrhage without trauma.

neonate / early childhood.

  • intracranial haemorrhage,
  • haematomas
  • cord bleeding in neonates.

Spontaneous bleeding into joints (haemoathrosis) and muscles (untreated this can lead to joint damage and deformity)

Gums
Gastrointestinal tract
Urinary tract causing haematuria
Retroperitoneal space
Intracranial
Following procedures
29
Q

haemophilia management

A

IV infusion of affected clotting factors (VIII or IX)
prophylactic or in response to bleeding

(complication is the formation of antibodies against the clotting factors)

Infusions of the affected factor (VIII or IX)
Desmopressin to stimulate the release of von Willebrand Factor
Antifibrinolytics such as tranexamic acid

30
Q

VWD management

A

in response to major bleeding or trauma and in preparation for ops.

Desmopressin can be used to stimulates the release of VWF
VWF can be infused
Factor VIII is often infused along with plasma-derived VWF

women:
Tranexamic acid
Mefanamic acid
Norethisterone
Combined oral contraceptive pill
Mirena coil
hysterectomy (severe)
31
Q

thrombophilia

A

predispose patients to develop blood clots

  • antiphospholipid syndrome
  • Antithrombin deficiency
  • Protein C or S deficiency
  • Factor V Leiden
  • Hyperhomocysteinaemia
  • Prothombin gene variant
  • Activated protein C resistance
32
Q

Budd-Chiari syndrome

A

blood clot develops in hepatic vein. blocks the outflow fo blood. associated with hypercoagulable state- causes acute hepatitis

triad:
Abdominal pain
Hepatomegaly
Ascites

manage: anticoagulation (heparin) (warfarin) investigate for underlying cause of hyper coagulation.

Haematology (14 decks)

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