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Haematology > Haemolytic Anaemia's > Flashcards

Haemolytic Anaemia's Flashcards

1
Q

1.Define Haemolytic Anaemia?

A

Anaemia caused by shortened RBC survival

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2
Q

2.Define Anaemia?

A

Reduced Hb level for the age and gender of the individual

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3
Q

3.List the 3 stages of an RBC’s life?

A
  1. RBC production
  2. Circulating RBC (120 days)
  3. Removal senescent RBC
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4
Q

4.Whats needed for the production of a RBC?

A
  • Iron
  • Erythropoietin (EPO)
  • B12/Folate
  • Globin Chains
  • Protoporphyrins
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5
Q

5.Explain 3 features of a mature RBC?

A
  • A biconcave disk as a membrane
  • Haemoglobin inside
  • Products of metabolic pathways inside (ie Glycolytic and hexose- monophosphate shunt pathway)
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6
Q

6.What is Haemolysis?

What causes Haemolysis?

A

Haemolysis= rupture of RBC’s
They have a shortened red cell survival (30-80 days), because of this the bone marrow compensates with increased RBC production
So there is increased young cells in circulation, this is called reticulocytosis and can be seen with + or - nucleated RBC

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7
Q

7.What is the difference between compensated haemolysis and incompletely compensated haemolysis?

A

Compensated Haemolysis –> RBC production able to compensate for decreased RBC life span—>Normal HB
Incompletely compensated Haemolysis = RBC production unable to keep up with decreased RBC life span—>decreased Hb

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8
Q

8.What are the clinical findings of Haemolytic Anaemia?

A
  • Jaundice (yellow)
  • Pallor (pale )/ Fatigue
  • Splenomagly (enlarged spleen)
  • Dark Urine
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9
Q

9.What is a Haemolytic crises?

A

Increased Anaemia –> Jaundice , infections /precipitants

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10
Q

10.What is Aplastic crises?

A

Anemia–> Reticulocytopenic with parvovirus infection

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11
Q

11.What are CHRONIC clinical findings of haemolytic anaemia?

A
  • Gallstones
  • Leg Ulcers
  • Folate Deficiency
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12
Q

12.What are the laboratory findings of Haemolytic anaemia?

A
  • Increased Reticulocyte count
  • Increased unconjugated bilirubin
  • Increased lactate dehydrogenase
  • Low serum haptoglobin (a protein that binds free Hb)
  • Increased urobilinogen
  • Increased urinary haemosiderin
  • Abnormal blood film
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13
Q
  1. Explain the appearance of each of the following on a blood film?
    - Reticulocytes
    - Polychromasia
    - Nucleated RBC
    - Poikilocytes
A

Reticulocytes = Have no clear white space in the middle , you can see the DNA strands
Polychromasia = multicoloured RBC’s all diff sizes and colours
Nucleated RBC= dark pigment (nucleus) seen inside RBC
Poikilocytes= They are abnormally shaped so like sickle shaped, oval shaped , tear drop etc..

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14
Q

14.When you have Haemolytic anaemia you have a risk of aplastic crises, from which virus?

A

Parvovirus B19

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15
Q

15.There are three categories of inherited Haemolytic anaemia’s
1. Membrane disorders
2. Enzyme disorders
3. Haemoglobin disorders
give 2 examples of each one :)

A

Membrane Disorders:

  • Spherocytosis
  • Elliptocytosis

Enzyme Disorders:

  • G6PD deficiency
  • Pyruvate kinase deficiency

Haemoglobin Disorders

  • Sickle cell anaemia
  • Thalassaemias
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16
Q

16.In what ways would you get acquired Haemolytic anaemia?

A

Immune
Drugs
Mechanical
Microangiopathic (disease of blood cells)
Infections
Burns
Paroxysmal nocturnal- hemoglobinuria ( Destruction of RBC’s by the complement system)

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17
Q

17.Explain Extravascular RBC breakdown?

A

Macrophage breaks down RBC

Broken down back into iron and bilirubin , breaks down globin chains Amino acid

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18
Q

18.Explain Intravascular RBC breakdown?

A

RBC released Hb
Hb free in blood and urine
Get iron in urine too

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19
Q

19.What are the 3 main components of a normal red cell membrane structure?

A
  1. Lipid bilayer
  2. Integral proteins
  3. Membrane skeleton
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20
Q

20.Within membrane disorders we can separate into vertical interaction and horizontal interaction defects
List some examples of defects in vertical interaction?

A
  • Spectrin
  • Band 3
  • Protein 4.2
  • Ankyrin
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21
Q

21.List some examples of defects in horizontal interaction?

A
  • Protein 4.1
  • Glycophorin C
  • Spectrin-HPP
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22
Q

22.Match:
-defects in vertical interaction
-defects in horizontal interaction
to
- hereditary spherocytes
-hereditary elliptocytosis

A 
VERTICAL = SPHEROCYTES
HORIZONTAL = ELLIPTOCYTOSIS
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23
Q
  1. The following questions refer to hereditary spherocytosis?
    - Is it common
    - In what fashion is it inherited
    - How is it caused?
    - What does it cause?
    - Appearance?
A

Common hereditary haemolytic anemia

Inherited in autosomal dominant fashion (75%)

Defects in proteins involved in vertical interactions between the membrane skeleton and the lipid bilayer
Decreased membrane deformability

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

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24
Q

24.What are the clinical features of hereditary spherocytosis?

A
  • No symptoms or severe haemolysis
  • Neonatal jaundice
  • Splenomegaly, pigment gallstones
  • Reduced esosin-5 maleimide (EMA) binding- binds to band 3
  • Positive family history
  • Negative direct antibody test
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25
Q

25.What 3 things would you do to manage hereditary spherocytosis?

A
  1. Monitor
  2. Folic acid transfusions
  3. Splenectomy
26
Q

26.What are the 3 metabolic pathways of RBC’s? And what is the purpose of the pathways?

A 
Pathways:
1.Glycolysis
2.Hexose-monophosphate shunt
3.Rapoport Luebering shunt
Purpose :
 To generate energy and maintain function in circulation
27
Q

27.What is the role of the hexose-monophosphate shunt?

A
  • Generates reduced glutathione

- Protects the cell from oxidative stress

28
Q

28.What are the effects of oxidative stress?

A
  1. Oxidation of Hb by oxidant radicals—> denatured Hb aggregates and forms Heinz bodies which bind to membrane —> Oxidised membrane proteins=Reduced RBC deformability
29
Q
  1. A part of the glycolytic pathway is glucose-6-phosphate dehydrogenase,the following questions refer to a deficiency of this
    - Is it hereditary , if so in what fashion is it inherited?
    - In what populations is it common?
    - What types of blood groups is it more severe in?
    - What is it’s range of clinical features?
A

Hereditary, X-linked disorder

Common in African, Asian, Mediterranean and Middle Eastern populations

Mild in African (type A), more severe in Mediterraneans (type B)

Clinical features range from asymptomatic to acute episodes to chronic haemolysis

30
Q

30.What are some features of G6PD?

What would you see on a blood film of a patient who has G6PD?

A

-Haemolysis (rupture of RBC)
-Reduced G6PD activity on an enzyme assay
-On a blood film :
Bite cells
Blister Cells and ghost cells
Heinz bodies (methylene blue)

31
Q

31.Why might G6PD activity appear as normal on an enzyme essay when it should be reduced in a patient with G6PD

A

Reticulocytosis

32
Q

32.What are the oxidative precipitants of G6PD?

A

-infections
-Fava/broad beans
-Drugs:
Dapsone
Nitrofurantoin
Ciprofloxacin
Primaquine

33
Q

33.What is pyruvate kinase required for?

A

Generating ATP

Membrane cation pumps

34
Q
  1. The following questions are about pyruvate kinase deficiency:
    - In what fashion is it inherited?
    - Whats its range of symptoms
    - What can it cause?
    - How would you treat it
A
  • Autosomal recessive
  • Membrane deformability–>Chronic anaemia
  • Can range from mild all the way to transfusion dependant
  • It improves with a splenectomy
35
Q

35.Briefly describe the structure of Haemoglobin

A

It is composed of a Haem group and globin.
The Haem group can be separated into Ferrous Iron (Fe++) and Protoporphyrin IX
The globin protein can be further broken down into 2alpha and 2beta globin.

36
Q

36.Out of chromosome 11 and chromosome 16, which one is Beta like and which one is alpha like?

A

Chromosome 11- Beta like

Chromsome 16- Alpha like

37
Q
  1. What components form:
    - HbA
    - HbA2
    - HbF
A 
HbA= 2 alpha, 2 beta
HbA2= 2 alpha, 2 delta
HbF= 2 alpha, 2 gamma
38
Q

38.During prenatal stage which globin gene is more prevalent? What is the clinical significance of this?

A

Early on in life you don’t make beta chains, mainly alpha chains
This is important because most diseases effect the beta chains so during this stage the only disease we need to worry about is alpha thalassamia 0- we need to make alpha chains in utero

39
Q

39.In terms of the production of globin genes
What is it called when you produce normal globin genes but the quantity is either too high or too low?
What do you get when you produce normal amounts but the globin genes are structurally abnormal?

A
  • Thalassamia’s

- Variant Haemoglobin’s

40
Q

40.Explain the consequences of mutations resulting in the variant haemoglobin’s:
HbS
Kb Koin
KbC

A

Hbs= Decreased Solubility, polymerisation- sickle shaped Hb

Kb Koin= Alter O2 binding and decreased stability. Results in Heinz body formation. Autosomal dominant

HbC= Decreased Solubility, crystallisation.Autosomal recessive

41
Q

41.How do you get Thalassaemias?

A
  • Imbalanced alpha and beta chains–> Excess of unpaired globin chains–>Unstable:
  • precipitate and damage RBC and their precursors
  • Ineffective erythropoiesis in bone marrow
  • Haemolytic anaemia
42
Q
  1. The following questions refer to beta thalassaemias
    - In what fashion are they inherited?
    - Are they more common or less common than alpha thalassaemias?
    - What kind of RBC’s would you see in a blood film?
    - What protective measures are put in place for newborns?
A

-Autosomal recessive
-More common
-Very little Hb inside RBC
-Offered anti-natal screening- blood test to see if you are a carrier “thalassamia trait”
All babies tested for sickle cell anemia at birth

43
Q

43.What are some key features would you be looking for to diagnose a thalassaemia trait?

A
  • Asymptomatic
  • Microcytic hypochromic anaemia (small paler RBC which lack Hb)
  • Increased RBC’s
  • HbA2 increased with beta thal trait
  • Low Hb,MCV, and MCH
44
Q

44.What is the diagnosis of thal trait often confused with?

A

Fe deficiency

45
Q

45.What are two ways to diagnose beta thal ?

A
  1. Globin chain synthesis (rarely done now)

2. DNA studies (Expensive)

46
Q

46.In what process would you diagnose an alpha trait?

A

Exclusion

47
Q

47.Beta thalassaemia major requires a transfusion in the 1st year of life. What are the consequences if not transfused?

A
  • Failure to thrive
  • Progressive hepatosplenomegaly (spleen and liver swell)
  • Bone marrow expansion – skeletal abnormalities
  • Death in 1st 5 years of life from anaemia
48
Q

48.What are some side effects of getting a blood transfusion?

A
  • Iron overload
  • Endocrinopathies (disease of an endocrine gland)
  • Heart failure
  • Liver Cirrhosis (liver tissue replaced with scar tissue -> non functioning liver)
49
Q

49.Explain the pathway from the mutation to having sickle cell anaemia?

A
  1. Point mutation in the beta globin gene . Glutamic Acid —> Valine
  2. Insoluble Hb tetramer when deoxygenated–>Polymerisation
  3. “Sickle” shaped cell
50
Q

50.List the various clinically significant sickling syndrome:

A 
HbSS
HbSC
HbS-D Punjab
HbS-O Arab
HbS-beta thalassaemia
51
Q

51.What are some acute complications of sickle cell disease?

A
  • Vitreous haemorrhage
  • Chest Syndrome
  • Splenic Sequestration
  • Haematuria”papillary necrosis”
  • Priapism
  • Aplastic Crisis
  • Leg Ulcers
52
Q

52.What are some chronic complications of sickle cell disease?

A
  • Stroke: ischemic & haemorrhagic
  • Cholecystitis
  • Hepatic Sequestration
  • Dactylitis
  • Bone pain and infarcts
  • Osteomyelitis
  • Retinal Detachment
53
Q

53.What are the clinical features of sickle cell disease? Acute Sickling? and Chronic Sickling effects

A
  • Silent Infarcts
  • Pulmonary Hypertension
  • Chronic lung disease, bronchiectasis
  • Erectile Dysfunction
  • Azoospermia
  • Chronic Pain Syndromes
  • Delayed puberty
  • Moya-Moya
  • Retinopathy, visual loss
  • Chronic renal failure
  • Avascular necrosis
  • Leg ulcers
54
Q

54.What are the laboratory findings of a patient with SCD?

A 
SCD:
-Painful crises
-Aplastic crises
-Infections
Acute Sickling:
-Chest Syndrome
-Splenic Sequestration
-Stroke
Chronic Sickling effects:
-Renal failure
-Avascular necrosis bone
55
Q

55.How would you confirm a diagnosis of SCA?

A
  • Anaemia ( Hb often 65-85)
  • Reticulocytosis
  • Increased NRBC - nucleated RBC
  • Raised Bilirubin
  • Low creatinine
56
Q

56.What is HPLC and how would you use it to diagnose SCA?

A

A solubility test:

  • Expose blood to reducing agent
  • HbS precipitated
  • Positive in trait and disease

Electrophoresis :
-Compare the size / structure of HbS against results

57
Q

57.Is HPLC a definitive test?

A

No

You would need to do a sickle solubility test

58
Q

58.What is the difference between autoimmune and alloimmune?

A

autoimmune - response to your own antigens

Alloimmune- Response to non self’s antigens of the same species

59
Q

59.What is immune haemolytic anaemia? How is it caused in an autoimmune sitiuation?

A

Your own antibody destroy your own RBC’s
Autoimmune usually idiopathic (spontaneous, unknown reason. because of igG and igM) Drug mediated, Cancer associated (LPDs- Lymphoproliferative disorders )

60
Q

60.How would immune haemolytic anaemia arise in a alloimmune situiation?

A
  • Transplacental transfer: a placenta transfer from mum to baby. ABO incompatibility or
  • Transfusion related: acute haemolytic transfusion related - ABO incompatibility or you get certain antigens like Rh groups or Duffy
61
Q

61.How would you get non-immune acquired haemolysis?

A

Paroxysmal nocturnal haemoglobinuria (rare acquired, life-threatening disease of the blood)

Fragmentation haemolysis , either mechanical or through Microangiopathic haemolysis which is caused by Disseminated intravascular coagulation (small blood clots in blood stream)and
Thrombotic thrombocytopenic purpura (clotting in small blood vessels –> low platelet count)

Other causes are severe burns and some infections eg malaria

Haematology (8 decks)

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