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Haematology > Haemolytic Anaemia > Flashcards

Haemolytic Anaemia Flashcards

1
Q

What is haemolytic anaemia?

A

Anaemia due to shortened RBC survival

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2
Q

What organ produces erythropoietin?

A

Kidneys produce erythroopooietin

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3
Q

What do RBC lose?

A

Lose their nucleus

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4
Q

What do RBC acquire as they get older and so what happens to them?

A

• As they get older, they aquire changes in membrane so are removed via spleen and liver

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5
Q

What components do RBC have?

A
  • Hb
  • Membrane
  • Enzymes that keep them running
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6
Q

What compensates with increased RBC production?

A

Bone marrow compensates with increased RBC production

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7
Q

What is reticulocytosis?

A

Its an increased number of young cells in circulation

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8
Q

What is compensated haemolysis?

A

Its RBC production which is able to compensate for decreased RBC life span

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9
Q

What is incompletely compensated haemolysis?

A

Its RBC production which is unable to keep up with the decreased RBC life span

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10
Q

What are the clinical findingd of haemolytic anaemia?

A
  • Jaundice
  • Pallor/fatigue
  • Splenomegaly
  • Dark urine
  • Aplastic-anaemia, reticulocytopenia with parvovirus infection
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11
Q

What is the spleen the site for?

A

The site for removal of abnormal cells so swells during anaemia

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12
Q

Why do we have dark urine in haemolytic anaemia?

A

Dark urine because the bilirubin comes out quickly in urine

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13
Q

What does the parvovirus infection cause in haemolytic anaemia?

A

Causes red cheeks and rash and turns off RBC production for 10 days

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14
Q

What are chronic clinical findings of haemolytic anaemia?

A

• Gallstones - pigment
• Leg ulcers (NO scavenging)
• Folate deficiency
-Increased use as RBCs are being made quickly

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15
Q

What are lab findings of haemolytic anaemia?

A 
• Increased reticulocyte count 
• Increased unconjugated bilirubin 
• Increased LDH (lactate dehydrogenase) 
	○ Non-specific, not just in anaemia
• Low serum haptoglobin
	○ Protein that binds free haemoglobin
• Increased urobilinogen
• Increased urinary haemosiderin
• Abnormal blood film
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16
Q

What does the blood film for haemolytic anaemia have present?

A
  • Reticulocytes
  • Polychromasia
  • Nucleated RBCs
  • Poikilocytes
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17
Q

What are the 3 factors we use to classify haemolytic anaemia?

A
  1. Inheritance
    - Inherited
    - Acquired
  2. Site of RBC destruction
    - Intravascular
    - Extravascular
  3. Origin of RBC damage
    - Intrinsic
    - Extrinsic
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18
Q

Inheritance of what can cause haemolytic anaemia?

A 
• Autoimmune haemolysis
	○ Can make antibodies against cells
• Membrane disorders
	○ Spherocytosis
	○ Elliptocytosis
• Enzyme disorders
	○ G6PD deficiency
	○ Pyruvate kinase deficiency
• Hb disorders
	○ Sickle cell anaemia
        ○Thalassaemias
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19
Q

What is the structure of a normal red blood cell?

A

○ Lipid bilayer
○ Integral proteins that anchor the membrane onto the cytoskeleton
§ Keep it stable
○ Membrane skeleton

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20
Q

What membrane disorders contribute to haemolytic anaemia?

A

○Defects in vertical interaction(spherocytosis)

○Defects in horizontal interaction(Eliptocytosis)

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21
Q

Defect in vertical interaction(Hereditary spherocytosis)

A 
○ Autosomal dominant
○ Spectrin
○ Band 3
○ Protein 4.2
○ Ankyrin
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22
Q

Defects in horizontal interaction(Hereditary elliptocytosis)

A

○ Protein 4.1
○ Glycophorin C
○ Spectrin – HPP

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23
Q

What is the most common hereditary haemolytic anaemia?

A

Hereditary spherocytosis

24
Q

What fashion is hereditary spherocytosis inherited in?

A

Inherited in autosomal dominant fashion

25
Q

What happens to RBC in hereditary spherocytosis?

A
  • Defects in proteins involved in vertical interactions between the membrane skeleton and the lipid bilayer
  • Decreased membrane deformability
26
Q

What does the bone marrow make in hereditary spherocytosis?

A

Bone marrow makes biconcave RBC, but as membrane is lost, the RBC become spherical

27
Q

What are the clinical features of hereditary spherocytosis?

A
  • Asymptomatic to severe haemolysis
  • Neonatal jaundice
  • Jaundice, splenomegaly, pigment gallstones
  • Reduced eosin-5-maleimide (EMA) binding – binds to band 3
  • Positive family hostory
  • Negative direct antibody test
28
Q

What is the management plan for hereditary spherocytosis?

A 
• Monitor
• Folic acid
• Transfusion
• Splenectomy
-The RBCs then have a remainder of the nucleus as seen on blood film because usually spleen responsible for removing nucleus
29
Q

What are the inherited enzymopathies involved in haemolytic anaemia?

A
  • G6P deficiency

* Pyruvate kinase deficiency

30
Q

What type of disorder is G6P deficiency?

A

X-linked disorder

31
Q

What do clinical features range from in G6P deficiency?

A

• Clinical features range from asymptomatic to acute episodes to chronic haemolysis

32
Q

What is the role of the HMP shunt?

A

-Generates reduced glutathione

33
Q

What does the HMP shunt do?

A

Protects the cell from oxidative stress

34
Q

What are the effects of oxidative stress?

A

○ Oxidation of Hb by oxidant radicals
○ Resulting denatured Hb aggregates & forms Heinz bodies – bind to membrane
○ Oxidised membrane proteins – reduced RBC deformability

35
Q

Features of G6P deficiency

A 
○ Haemolysis
○ Film:
	§ Bite cells
	§ Blister cells & ghost cells
	§ Heinz bodies (methylene blue)
○ Reduced G6PD activity on enzyme assay
         §May be falsely normal if reticulocytosis
36
Q

What is pyruvate kinase required for?

A

Required to generate ATP

37
Q

What fashion is pyruvate kinase inherited in?

A

Autosomal recessive

38
Q

What does pyruvate kinase deficiency cause?

A

Causes chronic anaemia

39
Q

What can chronica anaemia be improved with?

A

Improves with splenectomy

40
Q

What can go wrong with haemoglobin structures?

A
  1. Quantitative

2. Qualitative

41
Q

What happens in thalassemias?

A

○ Imbalanced alpha and beta chain production

○ Excess unpaired globin chains are unstable

42
Q

What do we always need for Hb?

A

Always need 2 alpha chains and 2 other like chains

43
Q

What does excess unpaired globin chains do?

A

○ Precipitate and damage RBC and their precursors

44
Q

What does excess unpaired globin chains lead to?

A

Lead to ineffective erythropoiesis in bone marrow

45
Q

What fashion in beta thalassaemia inherited in?

A

Autosomal recessive

46
Q

What is the diagnosis of thalassaemia trait?

A 
• Asymptomatic
• Microcytic hypochromic anaemia
• Low Hb, MCV, MCH
• Increased RBC
• Often confused with Fe deficiency
• HbA2 increased in b-thal trait –(diagnostic)
• a-thal trait often by exclusion
• globin chain synthesis (rarely done now)
DNA studies (expensive)
47
Q

What happens if patient suffering from beta thalassaemia isn’t transfused?

A

• If not transfused:
○ Failure to thrive
○ Progressive hepatosplenomegaly
○ Bone marrow expansion – skeletal abnormalities
○Death in 1st 5 years of life from anaemia

48
Q

What are side effects of transfusion?

A

○ Iron overload
§ Endocrinopathies
§ Heart failure
§ Liver cirrhosis

49
Q

What mutation causes sickle cell disease?

A

Point mutation in the β globin gene: glutamic acid → valine

50
Q

What causes the sickle shape to form in SCD?

A

Insoluble haemoglobin tetramer when deoxygenated leads to polymerisation leading to sickle shaped cells

51
Q

What are the clinical features of SCD?

A 
○ Painful crises
○ Aplastic crises
○ Infections
○ Acute sickling:
	§ Chest syndrome
	§ Splenic sequestration
Stroke
52
Q

What are chronic sickling effects of SCD?

A

○ Renal failure

○ Avascular necrosis bone

53
Q

What are the features of SCD in the lab?

A 
○ Anaemia
	§ Hb often 65-85
○ Reticulocytosis
○ Increased NRBC
○ Raised bilirubin
○ Low creatinine
54
Q

Autoimmune haemolysis

A

Idiopathic

55
Q

Alloimmune haemolysis

A
  • Transplacental transfer

- Transfusion related

56
Q

Non-immune acquired haemolysis

A
  • Paroxysmal nocturnal haemoglobinuria
  • Fragmentation haemolysis
  • Severe burns
  • Infections

Haematology (6 decks)

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