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Haemolytic Anaemia Flashcards

1
Q

What is haemolytic anaemia?

A

Anaemia due to shortened RBC survival

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2
Q

How long do RBCs circle for?

A

Approx 120 days

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3
Q

Where are senescent RBCs removed?

A

Reticular endothelial system of the liver and spleen

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4
Q

How long do haemolytic anaemia RBCs survive for?

A

30-80 days

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5
Q

What does the shortened red cell survival mean for the blood?

A

Increased RBC production and increased young cells in circulation -> reticulocytosis

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6
Q

What is incomplete compensated haemolysis?

A

When RBC production is unable to keep up with decreased RBC lifespan -> decreased haemoglobin

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7
Q

What are the symptoms of haemolytic anaemia?

A 
Jaundice
Pallor
Fatigue
Splenomegaly
Gallstones
Leg ulcers
Folate deficiency line folate use in RBC production
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8
Q

What would a peripheral blood film show in haemolytic anaemia?

A

Polychromatophilia
Nucleated RBC
Thrombocytosis
Neutrophilia with left shift

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9
Q

What happens to the bone marrow in haemolytic anaemias?

A

Erythroid hyperplasia

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10
Q

What does haemolytic anaemia cause in the bone marrow?

A

Increase in unconjugated bilirubin, lactate dehydrogenase, urobilinogen and urinary haemosiderin

Decrease serum haploglobin protein that binds free haemoglobin

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11
Q

What does erythroid hyperplasia cause?

A

Normoblastic reactions
Reversal of myeloid: erythroid ratio
Variable reticulocytosis

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12
Q

What are the general classifications of haemolytic anaemia?

A

Inheritance
Site of RBC destruction
Origin of RBC damage

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13
Q

What are the inheritance classifications of haemolytic anaemia?

A

Hereditary

Acquired

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14
Q

What are the site of RBC destruction classifications of haemolytic anaemia?

A

Intravascular

Extravascular

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15
Q

What are the origin of RBC damage classifications of haemolytic anaemia?

A

Intrinsic

Extrinsic

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16
Q

What is an example of hereditary haemolytic anaemia?

A

Hereditary spherocytosis

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17
Q

What is an example of acquired haemolytic anaemia?

A

Immune haemolytic anaemia

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18
Q

What is an example of intravascular haemolytic anaemia?

A

Haemolytic transfusion reaction

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19
Q

What is an example of extravascular haemolytic anaemia?

A

Autoimmune haemolysis

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20
Q

What is an example of intrinsic haemolytic anaemia?

A

G6PD deficiency

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21
Q

What is an example of extrinsic haemolytic anaemia?

A

Delayed haemolytic transfusion reaction

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22
Q

What are examples of membrane defect intrinsic haemolytic anaemia?

A

Hereditary spherocytosis, elliptocytosis or pyroporkilocytosis

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23
Q

What are examples of enzyme defect intrinsic haemolytic anaemia?

A

G6PD, PK

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24
Q

What are examples of haemoglobin defect intrinsic haemolytic anaemia?

A

Sickle cell

Thalassaemias

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25
What are examples of immune mediated extrinsic haemolytic anaemia?
Autoimmune (warm, cold, drug induced) | Alloimmune (HDN, haemolytic transfusion reaction)
26
What are examples of non-immune extrinsic haemolytic anaemia?
Red cell fragmentation syndromes Drugs Infections Hypersplenism
27
What normally happens when RBCs breakdown?
Macrophages break them down and they go to the liver to be excreted from the kidney
28
What happens in haemolytic anaemia for RBC breakdown?
RBCs lyse in veins, which leads to haemoglobinurea
29
Which integral proteins are normally found in a cell membrane?
Glycophorin A and C | Bard 3
30
What are the cytoskeletal proteins found in the RBC membrane?
Ankyrin Alpha and beta spectrin Protein 4.2 and 4.1 Actin
31
What type of disease is hereditary spherocytosis?
Autosomal dominant
32
What is hereditary spherocytosis caused by?
Defects in spectrin, ankyrin and protein 4.2
33
How is hereditary spherocytosis managed?
Monitoring Folic acid Transfusion Splenectomy
34
What do hereditary spherocytotic cells look like?
Deeply stained with no central pallor
35
What causes hereditary elliptocytosis?
Defects in alpha or beta spectrin Or Loss of interaction between ankyrin and spectrin or 4.1
36
What do hereditary elliptocytotic cells look like?
Elongated RBCs with no pointy ends
37
What are the clinical features of hereditary spherocytosis?
Neonatal jaundice Splenomegaly Pigment gallstones
38
What tests for hereditary spherocytosis?
Flow cytometry showing reduced eosin-5-maleimide binding
39
What happens if there is asymptomatic hereditary spherocytosis?
Severe haemolysis
40
What is the role of the HMP shunt in the pentose phosphate pathway?
Generates NADPH and reduced gluthathione | Protects the cell from oxidative stress
41
What genetic type of disease is a glucose-6-phosphate dehydrogenase deficiency?
X-linked
42
What is the cell morphology in G6P dehydrogenase deficiency?
Bite cells Blister cells and ghost cells Heinz bodies
43
What are the effects of a G6P dehydrogenase deficiency?
Oxidative stress | -> oxidation of Hb by oxidant radicals and oxidised membrane proteins
44
What does Hb oxidation by oxidant radicals result in?
Denatured Hb aggregates and forms heinz bodies that bind to the membrane
45
What do oxidised membrane proteins do?
Reduced RBC deformability
46
What are patients with G6P dehydrogenase deficiency advised to do (and why)?
Avoid oxidative drugs because they can cause oxidative stress
47
What do defects in pyruvate kinase affect?
RBC shape and deformability and the intracellular cation core
48
What are the general issues in thalassaemias?
Defect in the rate of synthesis of alpha or beta globin chain
49
Why is it an issue if there’s a defect in the alpha or beta globin chain production?
Excess unpaired globin chains are unstable -> heterogenous genetic disorders -> ineffective erythropoeisis
50
What are the types of thalassaemia?
``` Hydrop foetalis Beta thalassemia major Beta Thalassaemia intermedia Beta Thalassaemia minor Alpha thalassaemias Alpha Thalassaemia minor ```
51
What are the clinical features of beta thalassaemia major?
``` Severe anaemia Progressive hepatosplenomegaly Facial bone abnormalities Mild jaundice Intermittent infections ```
52
What are the peripheral blood cell symptoms of beta thalassaemia major?
``` Microcytic hypochromic cells Anisopoikilocytosis Target cells Nucleated RBCs Teardrop cells ```
53
What are the peripheral blood count symptoms of beta thalassaemia major?
Decreased MCV, MCH, MCHC
54
What is thalassaemia intermedia?
Disorder with a clinical manifestation between thalassaemia major and minor
55
What are the signs of beta thalassaemia intermedia?
Transfusion independent Diverse clinical phenotype Varying symptoms Increased bilirubin level
56
How do you diagnose thalassaemia intermedia?
Largely clinically
57
What are the symptoms of thalassaemia minor?
Asymptomatic
58
What is thalassaemia minor often confused with?
Iron deficiency
59
How do you diagnose thalassaemia minor?
Finding that HbA2 is increased in beta thalassaemia
60
What causes Hb barts hydrops syndromes?
Deletion of all four globin genes
61
How long do patients with Hb barts hydrops syndrome live for?
They don’t
62
What causes HbH disease?
3/4 alpha globin chains deleted
63
Where is HbH common?
SE asia
64
What are the clinical features of HbH disease?
Hepatosplenomegaly
65
What do you use to diagnose HbH disease?
Electrophoresis
66
What are the two types of alpha thalassaemias?
HbH disease and Hb barts hydrops syndrome
67
What are the features of blood cells in HbH disease?
Hypochromic microcytic Poikilocytosis Polychromasia Target cells
68
What are the features of alpha thalassaemia minor?
Normal or mild HA | MCV and MCH low
69
What does ‘sickle cell disease’ refer to?
All diseases as a result of inherited HbS
70
What is HbS caused by?
Single nucleotide substitution
71
What are the clinical symptoms of sickle cell disease?
Painful crises Aplastic crisis Frequent Infections
72
Why do sickle cell patients have frequent infections?
Hyposplenism
73
What are the clinical symptoms of acute sickling in sickle cell anaemia?
Chest syndrome Splenic sequestration Stroke
74
What are the clinical symptoms of chronic sickling in sickle cell anaemia?
Renal failure | Avascular bone necrosis
75
What are the sickle cell signs in the lab?
``` Anaemia (Hb 60-90) Reticulocytosis Increased NRBC Raised bilirubin Low creatine ```
76
How do you confirm a sickle cell diagnosis?
Solubility test: - Expose blood to a reducing agent - HbS precipitated

MBD/MI (Wothaya) (57 decks)

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