Practice Test #2 Flashcards

1
Q

What kind of therapy is needed for treating anaemia resulting from renal disease or from various other causes

A

Recombinant erythropoietin

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2
Q

What is the method of administration for Recombiant erythropoietin

A

Subcutaneously

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3
Q

How often is Recombinant erythropoietin used in treatment

A

three times weekly or once every 1–2 weeks or every 4 weeks

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4
Q

What is the main indication for Erythropoietin therapy

A

End-stage renal disease (with or without dialysis)

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5
Q

What is also needed in erythropoietin therapy

A

oral or intravenous iron

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6
Q

True or False, A high serum erythropoietin level prior to treatment is valuable in predicting an e ective response

A

False, Low Level

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7
Q

What are some common side effects for recombinant erythropoietin therapy

A

Side‐effects include a rise in blood pressure, thrombosis and local injection site reactions.

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8
Q

What hormones are necessary for effective erythropoiesis

A

Androgens and thy- roxine

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9
Q

What metals are needed in successive erythropoietin synthesis

A

iron and cobalt

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10
Q

What is the dominant haemoglobin in blood after the age of 3–6 months) c

A

HbA

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11
Q

What are the 4 polypeptide chain in Haemoglobin A

A

α2 β2, each with its own haem group

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12
Q

What are the clinical uses of erythropoietin

A

Anaemia of chronic renal disease
Myelodysplastic syndrome
Anaemia associated with malignancy and chemotherapy
Anaemia of chronic diseases, e.g. rheumatoid arthritis
Anaemia of prematurity
Perioperative uses

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13
Q

What is the main site for the protoporphyrin synthesis

A

Mitochondria

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14
Q

Where is Iron supplied from for erythropoiesis

A

Circulating transferrin

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15
Q

What synthesis occurs largely in the mitochondria

A

Haem

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16
Q

What is the key rate‐limiting enzyme in Haem Synthesis

A

δ‐aminolaevulinic acid (ALA) synthase

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17
Q

What is the coenzyme for Haem synthesis

A

Pyridoxal phosphate (vitamin B )

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18
Q

The condensation of which two compounds occurs in the biochemical reaction for haem synthesis

A

condensation of glycine and succinyl coenzyme A

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19
Q

The combination of which two compounds for Haem

A

Protoporphyrin combines

with iron in the ferrous (Fe2+)

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20
Q

True or False the contact of α1α2 β2β1 stabilises the structure of Haemoglobin

A

False it is, α2β2, α1β1

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21
Q

When O2 is unloaded β chains are pulled apart permitting the entry of which metabolite

A

2,3‐diphosphoglycerate (2,3‐DPG)

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22
Q

True or False, The entry of the metabolite in the β chains results in higher affnity of the molecule for O2.

A

False, lower

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23
Q

What is the P50 (i.e. the partial pressure of O2 at which haemoglobin is half saturated with O2) of normal blood

A

26.6 mmHg

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24
Q

What occurs with an increased affnity for O2 in a haemoglobin oxygen (O2) dissociation curve

A

Curve shifts to the left

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25
Q

True or False, The P50 will rise when there is an increase affinity for O2

A

False, it will fall

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26
Q

What causes the curve to shift to the right in a haemoglobin oxygen (O2) dissociation curve

A

Decreased affinity for O2

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27
Q

A rise in P50 is as result of

A

Decreased affinity for O2

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28
Q

In vivo, O2 exchange operates at ____ saturation in arterial blood

A

95%

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29
Q

in vivo, What is the mean arterial O2 tension

A

95mmHg

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30
Q

In vivo, O2 exchange operates at ____ in venous blood

A

70%

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31
Q

In vivo, What is the mean venous O2 tension

A

40 mmHg

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32
Q

The normal position of the curve depends on which 3 compound in the red cell

A

2,3‐DPG, H+ ions and CO2

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33
Q

What is the difference between normal Adult Hb and Fetal Hb

A

Fetal Hb is unable to bind 2,3 DPG

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34
Q

Rare abnormal haemoglobins associated with polycythaemia, will cause the oxygen dissociation curve to shift to which direction

A

Left

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35
Q

What is the name of the clinical state in which circulating haemoglobin is present with iron in the oxidized (Fe3+) instead of the usual Fe2+ state.

A

Methaemoglobinaemia

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36
Q

Where does Methaemoglobinaemia arise from

A

hereditary deficiency of methaemoglobin reductase deficiency or inheritance of a structurally abnormal haemoglobin (Hb M)

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37
Q

What illness is likely to show in patients with Methaemoglobin

A

Cyanosis

38
Q

What process does Haemoglobin use to generate ATP

A

anaerobic glycolytic (Embden–Meyerhof) pathway

39
Q

What is glucose metabolised to through facilitated transfer in RBC

A

Lactate

40
Q

What is generated in the breakdown of Glucose

A

NADPH, NADP

41
Q

For every Glucose molecule used, How many ATP molecules are generated

A

2

42
Q

The energy generated is used for the maintenance of what in RBC

A

Shape, flexibility, volume

43
Q

What is the enzyme methaemoglobin reductase used for

A

Reduce functionally dead methaemoglobin containing ferric iron, to functionally active, reduced haemoglobin containing ferrous ions

44
Q

In Embden–Meyerhof pathway, Luebering–Rapoport shunt, or side arm, of this pathway generates what metabolite

A

2,3‐DPG

45
Q

What pathway facilitates appx 10% of the glycolysis

A

Hexose monophosphate (pentose phosphate) shunt

46
Q

What is linked with NADPH in Hexose monophosphate (pentose phosphate) shunt, to maintain sulphydril SH groups

A

Glutathione

47
Q

What is the oxidative pathway for Hexose monophosphate (pentose phosphate) shunt

A

glucose‐6‐phosphate is converted to 6‐ phosphogluconate and so to ribulose‐5‐phosphate

48
Q

The deficiency of what substance causes red cells to be extremely susceptible to oxidant stress

A

glucose‐6‐phosphate dehydrogenase

49
Q

What process is used to number red cell proteins based on their mobility

A

Polyacrylamide gel electro- phoresis (PAGE)

50
Q

What structural proteins make up the membrane skeleton in RBC

A

α and β spectrin, ankyrin, protein 4.1 and actin

51
Q

What is described as a reduction in the haemoglobin concen- tration of the blood below normal for age and sex

A

Anaemia

52
Q

What is the normal value of haemoglobin in adult males

A

less than 135g/L in adult males

53
Q

What is the normal value of haemoglobin in adult females

A

115 g/L in adult females

54
Q

From the age of 2 years to puberty, less than how much Hb indicates anaemia is present

A

less than 110g/L

55
Q

What are the main causes of Anaemia

A

iron deficiency (hookworm, schistosomiasis), sickle cell diseases, thalassaemia, malaria and the anaemia of chronic disorders

56
Q

True or False, Rapidly progressive anaemia causes less symptoms than anaemia of slow onset

A

False, causes more symptoms

57
Q

Thee presence or absence of clinical features can be considered under what four major headings.

A

Speed of Onset, Severity, Age, Haemoglobin O2 dissociation curve

58
Q

What are the different signs of Anaemia for

a) iron deficiency
b) haemolytic or megaloblastic anaemia
c) sickle cell
d) thalassaemia major

A

a) Koilonychia (spoon nails) with iron deficiency,
b) jaundice with haemolytic or megaloblastic anaemias,
c) leg ulcers with sickle cell and other haemolytic anaemias, d) bone deformi- ties with thalassaemia major.

59
Q

What are the three different classifications of anaemia

A

microcytic, normocytic and macrocytic

60
Q

True or False, The Mean Corpuscular Volume (MCV) is usually high in newborns but low in infancy

A

True

61
Q

True or False, in normal pregnancy there is a decrease in MCV levels

A

False, there is an increase

62
Q

Thalassaemia
Anaemia of chronic disease (some cases)
Lead poisoning
Sideroblastic anaemia (some cases) is associated with which classification of Anaemia

A

Microcytic

63
Q

After acute blood loss
Renal disease
Mixed deficiencies
Bone marrow failure (e.g. post‐ chemotherapy, infiltration by carcinoma, etc.) is associated with which classification of anaemia

A

Normocytic, normochromic

64
Q

Megaloblastic: vitamin B12 or folate deficiency
Non‐megaloblastic: alcohol, liver disease, myelodysplasia, aplastic anaemia, etc. is associated with which type of anaemia

A

Macrocytic

65
Q

MCH <27pg

A

Microcytic, hypochromic

66
Q

MCV 80-95fL

A

Normocytic, normochromic

67
Q

MCV >95fL

A

Macrocytic

68
Q

MCH ≥27pg

A

Normocytic, normochromic

69
Q

MCV <80fL

A

Microcytic, hypochromic

70
Q

The laboratory finding of what two substances aid in distinguishing ‘pure’ anaemia from ‘pancytopenia’ (subnormal levels of red cells, neutrophils and platelets)

A

Leucocyte and platelet counts

71
Q

What is the normal Reticulocyte count and percentage

A

0.5–2.5%, 50–150 × 109/L

72
Q

True or False, The higher the Reticulocyte count, indicates the more severe of anaemia

A

True

73
Q

How long does it take for an erythropoietin response after an acute major haemorrhage

A

6 hours

74
Q

How long does it take before the reticulocyte count raises

How long does it take before the the reticulocyte reaches its maximum

A

2-3 days

6–10 days

75
Q

If the reticulocyte count is not raised in an anaemic patient, what does it suggest.

A

impaired marrow function or lack of erythropoietin stimulus

76
Q

What red cell abnormality is formed from Oxidant damage– e.g. G6PD deficiency, unstable haemoglobin

A

Basket cell

77
Q

What is examination is done when the cause of anaemia or other abnormality of the blood cells cannot be diagnosed from the blood count, lm and other blood tests alone

A

Bone marrow examination

78
Q

What are the two methods for bone marrow examination done

A

aspiration or trephine biopsy

79
Q

In aspiration of bone marrow, what is used to stain the liquid sample

A

Romanowsky technique

80
Q

What is usually observed in the liquid aspiration samples

A

the proportion of the different cell lines assessed

81
Q

True or false, trephine biopsy specimen is less valuable than aspiration when individual cell detail is to be examined

A

True

82
Q

Which bone marrow examination, provides more of a panoramic view of the marrow

A

trephine biopsy

83
Q

Which bone marrow examination, shows presence of fibrosis or abnormal infiltrates, with immunohistology

A

trephine biopsy

84
Q

Why is Erythropoiesis term ineffective

A

Because 10%-15% of developing erythoblasts die within the marrow before reaching mature cells

85
Q

What two substances facilitate the breakdown of Haemoglobin

A

Unconjugated Bilirubin. lactate dehydrogenase (LDH)

86
Q

What is the site of Bone Marrow investigation

A

Posterior iliac rest

87
Q

What stain is used in Trephine Biopsy

A

Haematoxylin and eosin; reticulin (silver stain)

88
Q

How long does it take for the Results to be available in

a) Aspiration
b) Trephine Biopsy

A

a) 1-2 hours

b) 1-7 days

89
Q

What does NADH prevent in haemoglobin

A

oxidation of haemoglobin

90
Q

What does NADPH prevent in haemoglobin

A

keeps cell proteins in the membrane and haemoglobin in the reduced state