Haem

Question 1

What is the management plan for Macrocytic Anaemia?

Answer 1

​Management depends on cause

• Pernicious Anaemia - IM Hydroxycobalamin for life
  
  • If no neurological defect = 1mg 3x/week for 2 weeks then 1mg/3months
  • If neurological defect = 1mg/daily until no more improvement then 1mg/2months
• B12 deficiency - severe - IM Hydroxycobalamin
  
  • ​moderate = oral hydroxycobalamin
  • mild/asymptomatic = dietary supplemets
• Folate deficiency - oral folic acid (always treat B12 first as B12 is needed to uptake folate)
  
  • ​In pregnancy, prophylactic folate is always given up till 12 weeks

Question 2

What is the management plan for Microcytic Anaemia?

Answer 2

• Iron deficient Anaemia
  
  • ​1st Line = oral iron (ferrous sulphate) w/ Ascorbic acid
  • 2nd Line = IV iron replacement –> RBC infusion if cardiac comprimise
• Sideroblastic Anaemia - treat cause e.g. alcohol cessation
  
  • ​pyroxidine - if inherited SA
  • Consider blood transfusion + iron chelation if no response
• Lead Poisoning - remove souce
  
  • ​Dimercaprol or D-penicillinamine

Question 3

What is the management plan for Sickle cell anaemia?

Answer 3

• 1st Line = supportive care and prevention of complications
  
  • ​If frequent painful crisis - Hydroxycarbamide (increases HbF)
    
    • ​prevents dactylitis and reduces stroke risk
    • If intolerant use L-glutamine
  • Crizanlizumab if >16 years to reduce painful crisis events
  • If severe = frequent blood transfusions + iron chelation
• 2nd Line = BM transplantation (may be curative)

Give advice to avoid precipitators and consider prophylactic ABx/vaccinations

Question 4

What is the management plan for Vaso-occlusive painful crisis in SCA?

Answer 4

• 1st Line = Analgesia (use WHO ladder) + O2 + Warmth + correct acidosis (sodium bicarbonate)
  
  • ​consider IV fluids, Abx + X-matched blood if necessary
• Acute chest syndrome - similar approach
  
  • ​2L/min O2 + incentive spirometry
  • Broad spectrum ABx