3: Haemolytic Anaemia Flashcards

1
Q

What is haemolytic anaemia

A

pre-mature destruction of RBC

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2
Q

What is the lifespan of a RBC

A

120-days

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3
Q

What are the two types of haemolytic anaemia

A

Intravascular haemolysis

Extravascular haemolysis

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4
Q

Where does intravascular haemolysis occur

A

Inside blood-vessels

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5
Q

Where does extravascular haemolysis occur

A

Outside blood-vessels

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6
Q

Aside from intravascular-extravascular how can anaemia be divided

A

Hereditary

Acquired

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7
Q

What are the 2 hereditary haemolytic anaemia due to enzyme defects

A
  • G6PD Deficiency

- Pyruvate Kinase Deficiency

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8
Q

What are the 2 hereditary haemolytic anaemia due to membrane defects

A
  • Hereditary spherocytosis

- Hereditary elliptocytosis

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9
Q

What are the 2 haemolytic anaemia due to haemaglobinopathies

A
  • Thalasemia

- Sickle cell

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10
Q

How can acquired haemolytic anaemias be divided

A

Immune mediated and non-immune mediated

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11
Q

If an immune mediated haemolytic anaemia, what will be positive

A

Coombs test (Direct antibody test)

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12
Q

what is a group of Coombs negative acquired haemolytic anaemia

A

Microangiopathic haemolytic anaemia

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13
Q

What is the inheritance pattern of G6PD deficiency

A

X-Linked recessive

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14
Q

How does G6PD present normally

A

Asymptomatic

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15
Q

What can precipitate a crisis in G6PD deficiency

A
  • anti-malarial
  • sulphur containing drugs: sulphonylurea, sulphasalazine,
  • ciprofloxacin
  • fava beans
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16
Q

How will G6PD crisis present

A

Jaundice
Gall stones
Splenomegaly

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17
Q

How may G6PD manifest in early days of life

A

Neonatal Jaundice

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18
Q

what is the most common RBC enzyme defect

A

G6PD Deficiency

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19
Q

which gender is G6PD more common in and why

A

Males - due to x-linked recessive

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20
Q

explain pathophysiology of G6PD

A

G6PD reduces glutathione. Deficiency therefore increases glutathione and oxidative stress leading to RBC breakdown and intravascular haemolysis

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21
Q

what will be seen on a blood film in G6PD

A
  • Heinz bodies
  • Bite cells
  • Blister cells
22
Q

explain management G6PD

A
  • avoid precipitants such as henna

- transfuse if severe

23
Q

what is the inheritance pattern of pyruvate kinase deficiency

A

Autosomal recessive

24
Q

how will patients with pyruvate kinase deficiency present

A

symptomatic:
- Jaundice
- Lethargy
- Splenomegaly
- Pruritus

25
Q

what is hereditary spherocytosis

A

autosomal dominant condition affecting RBC cytoskeleton causing it to form a spherical, opposed to bi con-cave, shape

26
Q

how does hereditary spherocytosis present clinically

A
  • Jaundice
  • Pigment gallstones
  • Splenomegaly
  • Aplastic crisis due to parvovirus B19
27
Q

if equivocal about diagnosis of HS, how is patient investigated

A

cryohaemolysis test and EMA binding

28
Q

how is HS managed

A
  • Folate

- Splenectomy

29
Q

how does hereditary elliptocytosis present

A

Autosomal dominant condition where there is defect in RBC causing it to form a spherical shape

30
Q

what is the advantage of hereditary elliptocytosis

A

Protective against malaria

31
Q

what are autoimmune haemolytic anaemia

A

Extravascular haemolysis mediated by auto-antibody binding

32
Q

what are autoimmune haemolytic anaemias characterised by

A

Temperature at which autoantibodies bind

33
Q

what are the 3 types of AIHA

A
  • Warm AIHA
  • Cold AIHA
  • Paroxysmal Cold Haemaglobinuria
34
Q

what antibody mediates warm AIHA and how can you remember this

A

IgG

Grace is a warm person

35
Q

what temperature do antibodies bind in warm AIHA

A

37’

36
Q

what type of haemolysis does warm AIHA lead to

A

Extravascular

37
Q

what will be seen on peripheral blood smear in warm AIHA

A

Spherocytes

38
Q

what test will be positive in warm AIHA

A

Coombs test

Direct antiglobulin test

39
Q

overall what can be given to control symptoms of warm AIHA

A

glucocorticoids

40
Q

how is warm AIHA managed in adults

A

splenectomy

41
Q

how is warm AIHA managed in children

A

rituximab

42
Q

what antibody mediates cold AIHA

A

IgM

43
Q

what temperature does IgM bind RBC in cold AIHA

A

Below 4’

44
Q

explain presentation of cold AIHA

A

Chronic anaemia worsened by the cold

45
Q

what test is positive in cold AIHA

A

Coombs test (Dat test)

46
Q

how is cold AIHA managed

A
  • Keep warm

- Chlorambucil

47
Q

what is paroxysmal cold haemaglobinuria

A

mediated by the donath Landsteiner antibody that binds RBC in the cold and then causes haemolysis on re-warming

48
Q

what antibody causes paroxysmal cold haemaglobinuria

A

donath landsteiner

49
Q

what is the stereotype for paroxysmal cold haemaglobinuria

A

young child playing in the cold then comes inside and is jaundiced and fatigued

50
Q

what is paroxysmal nocturnal haemaglobinuria

A

rare disorder where there is haemolysis at night leading to marrow failure

51
Q

what is indicative of PNH

A

urinary haemosiderin