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Advanced Haematology > Week 1 > Flashcards

Week 1 Flashcards

1
Q

What are the 3 different mechanisms of action of Erythropoietin?

A
  1. Early release of reticulocytes.
  2. Inhibition of apoptosis
  3. Reduce processing time of blood cells. (released faster)
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2
Q

RBC’s consist of how many globin chains and how many pairs?

A

4 globin chains and 2 pairs.

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3
Q

Each globin has how many heme groups?

A

1

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4
Q

Does ferrous iron (Fe2+) combine irreversibly or reversibly with oxygen?

A

reversibly

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5
Q

Majority of RBC natural death occurs in the ________.

A

Spleen - extravascular macrophage mediated haemolysis.

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6
Q

When it comes to investigating haemolysis what are you looking for?

A

increased RBC destruction, increased RBC produciton, evidence of damaged RBC’s

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7
Q

Where does Intravascular and extravascular haemolysis take place?

A

Intravascular - within blood vessels

Extravascular - outside the blood vessels

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8
Q

Haemoglobinaemia (increased plasma Hb) decreased haptoglobin, increase plasma bilirubin (unconjugated, haemoglobinuria, haemosiderinuria (iron in renal tubules) are all evidence of extravascular RBC destruction or intravascular RBC destruction?

A

Intravascular

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9
Q

What is the normal way of removing majority of senescent RBC’s?

A

macrophage mediated (extravascular) haemolysis

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10
Q

Increased extravascular destruction can happen when RBC’s have abnormal markers and are removed. What are 3 examples of abnormal markers on RBC’s?

A

heinz bodies
intracellular parasites
immunoglobulins

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11
Q

What would be seen on a blood film if a macrophage ingested only part of RBC membrane?

A

Spherocyte - Remainder membrane seals gap with ↓ content

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12
Q

Is this evidence of intravascular or extravascular RBC destruction? increased urobilinogen (urine), increased plasma bilirubin, increased stercobilinogen (faecal) decreased haptoglobin.

A

Extravascular

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13
Q

What do intrinsic and extrinsic RBC defects refer to.

A

Intrinsic - in the RBC itself

Extrinsic - the environment surrounding the RBC

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14
Q

Give 3 examples of intrinsic defects.

A 
Membrane abnormalities (eg. spherocytosis)
Enzymopathies( G6PD deficiency)
haemoglobinopathies (sickle cell, thalassemias)
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15
Q

What are some laboratory findings for hereditary spherocytosis?

A 
Anaemia = yes normally
Retic = normally increased
MCV & MCH = normal
MCHC = increased
Blood film = spherocytes 
RDW= increased
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16
Q

List 3 other tests to determine hereditary spherocytosis

A
  1. DAT
  2. Osmotic fragility test
  3. Autohaemolysis test
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17
Q

What does a neg DAT mean?

A

No Antibody/s bound to RBC

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18
Q

What DAT result would you expect in a patient with hereditary spherocytosis?

A

Neg

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19
Q

What does the DAT determine?

A

If RBC’s have been sensitised in vivo. Is there an antibody bound to the RBC

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20
Q

Describe the osmotic fragility test.

A

RBC’s are added to increasing hypotonic NaCl solutions. H2O enters cell until equilibrium is achieved. Cell swells until membrane cannot withhold the pressure

21
Q

In the osmotic fragility test what would you expect a patient with hereditary spherocytosis to have?

A

RBC lysis beginning earlier in lower concentrations of the hypotonic NaCl solutions.

22
Q

Describe an autohaemolysis test.

A

RBC are incubated in own plasma. RBC’s lyse gradually. (5% after 48 hours) more if glucose is added

23
Q

What would the result of an autohaemolysis test be in a patient with hereditary spherocytosis?

A

haemolysis is much greater after 48hr (10-50% lysis)

24
Q

Is eliptocytosis and ovalocytosis more common in malria endemic areas?

A

Yes

25
Q

What are 2 most common enzymopathies?

A

G6PD - deficiency

Pyruvate Kinase deficieny.

26
Q

List three triggers of oxidative stress?

A
  1. Infection (most common)
  2. Some drugs
  3. Fava beans
27
Q

What are Heinz bodies and how do you visualise Heinz bodies?

A

Denatured Hb seen with a supravital stain eg. crystal violet

28
Q

Haemoglobinopathies are all due to mutations in what?

A

Gene mutation

29
Q

Haemoglobinopathy that has a structural defect is

A

Sickle cell disease = quality of Hb

30
Q

Haemoglobinopathy that has a reduced rate of synthesis

A

Thalassemia = defect in quantity

31
Q

Describe why a patient with sickle cell disease might experience joint pain

A

Sickle RBC’s are less soluble & less deformable, more fragile, leads to haemolysis, leads to vessel occlusion leading to infarct. Increase susceptibility to infection.

32
Q

List other screening tests for sickle cell disease.

A
  1. Hb electrophoresis

2. Haemoglobin solubility test

33
Q

What would be the difference between sickle cell trait and sickle cell anemia on an electrophoresis test?

A

Sickle cell anemia would be all HbS. Sickle cell trait would HbS plus another Hb beta chain.

34
Q

What is the most severe form of Thalassemia?

A

Beta thal major

35
Q

Would the MCV be low or high in a patient with Thalassemia?

A

Low

36
Q

Blood film of a patient with thalassemia could include.

A 
Target Cells
Elliptocytes
other poikilocytes
basophilic stippling
nucleated RBC (major thal)
37
Q

Alloantibodies means

A

Antibodies to foreign

38
Q

Autoantibodies means

A

Antibodies to self

39
Q

Besides malaria what are some other causes of non-immune RBC haemolysis?

A

Drugs, chemicals, venoms, burns

40
Q

What are the two types of Auto antibody?

A 
Warm autoimmune (most common)
Cold agglutinins
41
Q

WARM AUTOimmune Haemolytic Anaemia (WAIHA) is what Ig class?

A

IgG

42
Q

Normal age group for WARM AUTOimmune Haemolytic Anaemia (WAIHA) is

A

under 4 and over 40

43
Q

What is the Ig class for COLD Agglutinin Disease (CAD)

A

IgM (4 degrees optimal)

44
Q

What can be a source of foreign Ag to cause ALLO immune Haemolytic Anaemia?

A

Blood transfusions

45
Q

How is Drug-Induced Immune Haemolytic Anaemia different to drug-induced haemolytic Anaemia?

A

in DIIHA drug us absorb to the RBC = immune complex - Ab is porduce against it - DAT will be positive

46
Q

List 3 conditions with small RBC’s and briefly discuss why the RBC’s are smaller

A

Thalassemia minor - reduced synthesis of the globin chain of hemoglobin
iron deficiency - disruption of iron supply
Sideroblastic anemia

47
Q

List 3 conditions with large RBC’s and briefly discuss why the RBC’s are larger

A

Megaloblastic anaemia
Alcoholism
Pernicious anaemia

48
Q

What is the most common cause of microcytic anemia?

A

iron deficiency

Advanced Haematology (13 decks)

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