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Haemotology > Sickle cell disease > Flashcards

Sickle cell disease Flashcards

1
Q

Why is sickle cell disease still present

A

Sickle cell trait provides protection against malaria in endemic areas, Sickle cell disease doesnt but occurs when inherit two faulty genes

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2
Q

What mutation causes sickle cell

A

Beta chain - glutamic acid replaced with valine (less polar)

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3
Q

Why does deoxygenation of RBC matter in sickle cell

A

When deoxygenated, confirmation exposes mutation -> less oluble and allows polymerisation -> vasoocclusion

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4
Q

What does polymerisation of RBC in sickle cell cause

A

Ischaemia and necrosis #Haemolysis of damaged RBC

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5
Q

Risk factors for sickle cell crisis

A

-Anything that shifts oxygen dissociation curve to the right - deoxy Hb
Hypoxia
Fever
Acidosis
Increased temp
Infection
Dehydration
-Anything -> vasoconstriction
Cold
Pain
Menstrual periods
Alcohol
Dehydration

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6
Q

What causes tissue damage in sickle cell

A

Ischaemia -> necorsis
Neurotrasnmitters and other mediators eg nociceptors
Intravascular haemolysis (RBC contents -> bloodstream)

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7
Q

Symptoms of sickle cell

A

PAIN - constant or intermittent
Sickle cell cirsis
Hyposlpenism -> increased risk of infections

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8
Q

Complications of sickle cell

A

Stroke
Renal disease, chronic HPTN
Priapism
Hyposplenism
Lung disease
Retinopathy
Fatigue
Miscarriage
AVN - bones??
Current life expectancy mid 60s

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9
Q

Treatment of sickle cell

A

Treat comlpications
Treat sickle crisis
Monitor and treat chronic complications -
-penicillin prophylaxis infection
Imunisations
Contol BP
Monitor pregnancies
Retinopathy screening
Hydrocycarbamide

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10
Q

How to reduce crisis in sickle cell

A

Hydroxyccarbamide
Regular transfusions
Bone marrow transplant - only cure

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11
Q

How does hydroxycarbamide work

A

Increases gamma chain production -> increased foetal haemoglobin

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12
Q

How does transfusion work in sickle cell? Complications?

A

Reduce sickle Hb in blood, less frequent crisis, doesnt stop haemolysis
Can cause iron overload AB formation

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13
Q

Bone marow transplant to treat sickle cell risk

A

Can cure but very high risk
Morality and morbidity high not always successful
V selective with who receives

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14
Q

New treatments for sickle cell

A

Gene therapy
Voxelotor - inhibit HbS polymerisation
Crizanilzumab - anti P selectin

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15
Q

Where is commonly affected in sickle cell crisis

A

Back, chest, extremeties

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16
Q

Sickle cell crisis symptoms

A

Pain
Mild fever, swelling of bones and joints
2-7 days

17
Q

What prophylaxis is given to all sickle cell crisis

A

DVT

18
Q

What investigation use to prevent progression to acute chest syndrome

A

Incentive spirometry

19
Q

What occurs in sickle trait and hwy

A

Mild haemolysis
Protective against malaria
Disease = RBC reduced lifespan massively and splenic dysfunction

20
Q

What does beta mutation cause in sickle cell

A

(glutamic acid replaced with valine in chain - valine less polar -> less soluble and polymerisation Hb

21
Q

Predisposing factors to sickle cell crisis

A

Anything shifting oxygen dissociation curve - deoxygenating RBCs
-hypoxia, fever, acidosis, increased temp, infection, dehydration
Anything -> casoconstriction
Cold, pain, dehydration

22
Q

Cycle of sickle cell

A

Vasoconstriction -> worsening vasocculsion and tissue hypoxia
Vasoocclusion -> tissue hypoxia and pain
Tissue hypoxia -> sickling -> haemolysis

23
Q

What to do sickle crisis first aid

A
  • Treat pain - escalate painkiller quickly, patient controlled useful
  • Hydrate - oral or IV
  • Keep warm
  • Treat infection
  • Consider oxygen if hypoxic (otherwise unhelpful)
  • Consider transfusion - haemotology
  • Consider secondary effects
    • Chest crisis - sickling lungs -. hypoxia, high mortality
    • Priapism
    • Stroke - thrombolysis
24
Q

What to monitor in sickle cell

A

Penicillin prophylaxis
Immunisations for hyposplenism
Control BP
Monitoring in oregnancy
Retinopathy screening

25
Q

How does hydroxycarbamide work

A

Increasing haema/gamma Hb (foetal) eg normal

26
Q

Reducing number of crisiss

A

Hydroxycarbamide
Regular blood transfusions in some patinet
Bone marrow transplant curative but c high risk
Oxelotor
Crianlizumab
Gene therapy

27
Q
A
27
Q

How does oxelotor and crizanlizumab work

A

Oxelotor - inhibit HbS polymerisation
Crizanlizumab - antiP selecion

28
Q

Complications of regular blood transfusions

A

Iron overloard, AB formation

29
Q

Diagnosis of sickle cell

A

Hb electrophoresisi

30
Q

Priapism what is it

A

Painful penile erection secondary to venous obstruction otuflow from corpus cavernosum

31
Q

INfe tions more likely in sickle cell

A

Strep pneumoniae, h influenzaen

32
Q

ocular comps sickle cell

A

Retinopathy
Vitreous haemorrhage
Retinal detatchement

33
Q

What can parvovirus B19 cause in sickle cell

A

Aplastic crisis - temproaraily halts production RBC

33
Q

Acute chest syndrome symptoms

A

Fever, chest pain, cough, SOB, hypoxia

34
Q

When does sickling crisis occur HbAS vs HbSS

A

HbAS - 2.5-4 pO2 kpa
HbSS - 5-6 pO2 Kpa

35
Q

Inheritance sickle cell

A

Autosomal recessive

36
Q

How often give pneumococcal vaccine in sicke cell

A

every 5 years

Haemotology (19 decks)

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