Revision Lectures

Question 1

Paroxysmal Nocturnal Haemoglobinuria

Answer 1

• Dark coloured urine in the morning
• Lack of glycoprotein - GPI
• Increased complement sensitivity
• Lack of CD59 = platelet aggreggation
• Haemolytic anaemia +/- pancytopenia
• Increased thrombosis
• Budd- Chiari syndrome (occlusion of the hepatic veins that drain the liver)

Question 2

Sickle cell Anaemia

Answer 2

• Autosomal recessive disease - heterozygous = trait
• Abnormal Hbs –> will “sickle” with low Pa02
• Symptoms develop at greater than 6 months old (HbF)
• Small bone infarction
• Kidney necrosis + tubular damage
• Autosplenectomy - spleen self disrtuction, normally silent. Therefore immunocompromised
• Sickle crisis –> sequestration in organs or thrombotic events.
• normally triggered by infection

Question 3

Investigations for anaemia?

Answer 3

Reduction in Hb or RBC insufficient for the bodies:

• FBC
• Iron studies
• Haemotinics
• Blood films

Question 4

Iron deficiency anaemia

Answer 4

• Insufficient intake
• Dietary
• malabsorption
• increased demands
• Excess loss
• bleeding; menstruation, GI bleed, bleeding disorders.

Treatment - iron supplementation = ferrous fumerate 210mg BD. Increase dietary intake - leafy greens, meat. Treat malabsorption.

Question 5

Do iron deficiency need investigations for malignancy?

Answer 5

• Endoscopy
• Colonoscopy
• CT CAP

Question 6

G6PD

Answer 6

• Nitrofurantoin is common trigger of crisis

Question 7

Thalassaemia trait

Answer 7

• Autosomal recessive
• Mild hypochromia, microcytic anaemia
• HbA2 raised >3.5%

Question 8

Beta Thalassaemia major

Answer 8

• Chr 11
• Presents in first year of life
• Splenomegaly, failure to thrive
• Requires lifelong blood transfusions (+ iron chelation)

Question 9

Alpha thalassaemia

Answer 9

• Chr 16
• Severity depends on how many chains affected
• 1 or 2 chains = hypochromic + microcytic (but Hb normal)
• 3 chains = HbH disease
• 4 chains = fetal death (hydrops)

Question 10

Anaemia of chronic disease

Answer 10

• Cause by ongoing inflammation in the body
• Common conditions = CKD, IBD, TB, cancer etc
• May have some overlap with iron deficiency anaemia -> look at ferritin

Question 11

B12 + Folate

Answer 11

• Blood film will show megaloblastic with hypersegmented neutrophil
• Intake through diet
• Multiple causes of deficiency: alcohol, methothrexate, reduced absorption
• Folate absorbed via jejunum
• B12 absorbed via ileum.
• Intrinsic factor released from parietal cells in stomach required for binding
• Pernicious anaemia = autoimmune condition with antibodies against the intrinsic factor
• Replace B12 before folate -> risk of subacute combined degeneration of the spinal cord.

Question 12

Aplastic anaemia

Answer 12

• Hypoplastic bone marrow
• Peak incidence ~30yrs
• Causes:
• idiopathic
• congenital
• drugs (phenytoin, oral chloramphenicol)
• viral
• radiation
• Pancytopaenia
• 10% develop acute leukaemia
• 7% Develop paroxysmal nocturnal haemoglobinuria
• Treatment: supportive, immunosuppressants, bone marrow transplant

Question 13

Lead poisoning

Answer 13

symptoms = Fatigue, constipation, struggling with hands and feel numb, blue line along the gums.

Finding = microcytic anaemia.

Question 14

Alcoholic anaemia

Answer 14

• High alcohol, no nutrients.
  Macrocytic anaemia
  macrocytosis (raised MCV but normal Hb) - indicates too much alcohol

If patient has:

1. Not eating + nutrients = B12 and folate therefore macrocytic megaloblastic picture.
2. Eating/corrected = alcohol can cause macrocytic non megaloblastic anaemia
3. liver disease = non megaloblastic macrocytic picture.

Question 15

Haemolytic anaemia

Answer 15

• Multiple causes; multiple ways of classifying.
• autoimmune
• inherited vs acquired
• intracorpuscular vs extracorpuscular
• haemoglobinopathies
• intravascular vs extravascualr
• Increased RBC breakdown
• Causing anaemia if there is insufficient turnover

Question 16

Haemolytic anaemia investigations:

Answer 16

• FBC + iron studies + Haematinics
• Blood film
• LFTs + bilirubin
• LDH
• Urine
• DAT (coombs)

Question 17

Autoimmune haemolytic anaemia

Answer 17

• Warm AIHA –> 37 degrees, IgG, Steroids/immunosuppressants
• Cold AIHA –> 4 degrees, IgM, avoid triggers.

NB: DAT/coombs test positive*

Question 18

G6PD

Answer 18

• Common exam question but rare condition
• Lack of G6PD which normally protects RBC from oxidative damage
• X-linked condition
• Sardinian, Kurdish Jews, Nigerian, Thai
• Presence of heinz bodies on blood film
• Haemolysis crisis precipitated by illness, medications + fava beans