Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Clinical Haematology > RBCs, Anaemia and Haemaglobinopathy > Flashcards

RBCs, Anaemia and Haemaglobinopathy Flashcards

1
Q

What is the average size of a red blood cell?

A

7um

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What shape do the RBCs take?

A

Biconcave discs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Blood pH is between ____ - ______

A

Blood pH is between 7.35-7.45

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Acidosis is a clinical condition where blood pH is below ____

A

7.35

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

The clinical symptoms of Acidosis are…

A

Headaches, lethargy, breathlessness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Some transmembrane proteins of the RBC lipid bilayer are…

A

Glycophorin C
Ankyrin
Spectrin
Tropomyosin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

The role of Glycophorin A is

A

maintenance of the negative charge, electrostatic repulsion to prevent adhesion, sugar transport

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

The role of band 3 protein is

A

anion transport, esp chloride and bicarbonate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

The role of glycophorin C is

A

Regulating cell shape, membrane deformability, membrane mechanical stability

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Ankyrin..

A

links lipid bilayer to spectrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Spectrin is responsible for the

A

maintenance of biconcave discs, they are the most abundant protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

RBCs metabolic activity is

A

Anaerobic glycolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Anaerobic glycolysis does:

A

maintain Haem Fe in the reduced state and reduces metHb by NADPH.
Produces ATP as a source of energy to maintain cell membrane deformability and realties ion exchange
production of 2,3-DPG to regulate O2 affinity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Dehydration of RBC leads to _____ cells

A

Dehydration of RBS leads to SPUTNIK cells

MetHB cannot carry O2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

The process of RBC production is called?

A

Erythropoiesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

In the Adult where does RBC production occur?

A

Bone marrow of sternum, pelvis and long bones

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Red cell production is regulated by _______ produced in the _______.

A

Red cell production is regulated by ERYTHROPOETIN produced in the KIDNEYS.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is the role of Erythropoietin?

A

Erythropoietin acts upon a committed ERYTHROID precursor to increase cell division

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

In renal hypoxia is erythropoietin production increase or decrease?

A

Increase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Immature RBC are nucleated True or False?

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is the lifespan of a RBC?

A

120 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

an increased reticulocyte count means..

A

an increased rate of RBC production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are senescent RBC killed by?

A

Macrophages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Where are senescent RBC killed?

A

In the Reticule endothelial system = SPLEEN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Macrophages line the ______ ______ in the spleen

A

SPLENIC CORDS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

The white pulp is the ..

A

primary lymphoid section

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what causes hastened destruction of RBC by splenic macrophages int he red pulp?

A

loss of membrane pliability or Ab coating

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

List the particle removal stages:

A
  1. Nuclear material (howell-jolly bodies)
  2. Cytoplasmic organelles
  3. Sideritic granules
  4. Oxidised Hb (MetHb)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

what is the weight of Hb in kDa?

A

68kDa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is the structure of Adult Hb? (HbA)

A

α2 β2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is the structure of HbA2 Hb?

A

α2δ2

32
Q

What is the structure of fatal Hb? (HbF)

A

α2γ2

33
Q

HbF has a _____ affinity for O2 than maternal HB, in order to ______ oxygen

A

HbF has a HIGHER affinity for O2 than maternal HB, in order to RECIEVE oxygen

34
Q

have a ____ O2 affinity where pO2 is _____, but a _____ affinity where pO2 is ____

A

Have a HIGH O2 affinity where pO2 is HIGH, but a LOW O2 affinity where pO2 is LOW

35
Q

2,3DPG binds preferentially to what state Hb?

A

deoxyHb, thus reducing O2 affinity

36
Q

What effect does acidosis have on O2 affinity?

A

reduces affinity, otherwise known as the BOHR EFFECT

37
Q

What causes the reduced affinity of HbF?

A

less active 2,3DPG

38
Q

What is the problem with free global chains?

A

they are toxic to the kidneys, scavenge nitrous oxide, leading to vasoconstriction and hypertension

39
Q

List some advantages of Artificial RBCs

A 
>storage temp &shelf life
>immediate universal application
>no lag in effectiveness
>no risk of disease transmission
>not dependant on donors
>avoid religious/cultural issues
40
Q

What is the definition of Anaemia?

A

Reduced concentration of Hb in the blood

41
Q

What are the symptoms of anaemia?

A 
Hb falls below 90-100g/L
slow onset
SOB
weakness
pallor
lethargy
palpitations
headaches
heart failure and confusion
42
Q

What are some clinical signs of anaemia?

A 
Concave nails
Jaundice
Leg ulcers
Bone deformations
Recurrent infection and/or bruising
43
Q

What is the normal Mean cell Volume? (MCV)

A

80-101fl

44
Q

What is the normal mean cell Hb (MCH) =Hb/RBC

A

27-34pg

45
Q

What is the normal Mean Cell Corpuscular Hb Concentration (MCHC)? =Hb/HcT

A

300-350g/L

46
Q

Classification of anaemia is by?

A

Impaired production compared with increased RBC loss/destruction, more usually by visual categorisation

47
Q

Normocytic, normochromic anaemia is when

A

RBC are of normal volume (MCV)
contain normal amount of Hb at the right conc (MCH,MCHC)
RBC count is reduced

48
Q

Give some examples of cases where NN anaemia can occur?

A

cute bleed, haemolysis, bone marrow failure, renal failure

49
Q

Microcytic, Hypochromic anaemia is when

A

RBCs are reduced in volume,

Recused Hb at a lower concentration than normal, (MCV,MCH,MCHC)

50
Q

what are some causes of Microcytic Hypochromic anaemia?

A

Fe deficiency
Thalassemia
Anaemia of Chronic disorder

51
Q

Microcytic, Normochromic Anaemia is when

A

RBC count is low,
RBS are increased in volume (MCV)
Hb is at normal concentrations (MCH increased, MCHC normal)

52
Q

Give some examples of where microcytic, normochromic anaemia can occur>

A

B12 or folate deficiency

53
Q

List the clinical causes of anaemia

A

1.RBC cytoskeletal disorder
2.Acquiired & inherited impairments of erythropoiesis
3.Acquired haemolytic anaemia
4.RBC enzymopathies
5.Haemoglobinopathy:
i)structural variants of Hb
II) Globin chain synthesis impairment

54
Q

Cytoskeletal RBC disorders include:

A

Hereditary elipocytes - disorder of spectrum, glycophorin C or protein 4.1 disorder)
NORMOCYTIC NORMOCHROMIC
Heridatory spherocytosis- >200-300 cases/10^6, caused by spectrum, ankyrin, band 3 or protein 4.1 disorder. many cases clinically silent, considerable genetic heterogeneity, increased osmotic fragility. NORMOCYTIC NORMOCHROMIC

55
Q

How would you treat hereditary spherocytosis?

A

removal of the spleen

Prophalytic AB treatment after surgery

56
Q

What are the causes of acquired impairment of erythropoiesis?

A

replacement of erythropoietic tissue by tumour as in metastes- NORMOCYTIC NORMOCHROMIC
Transient failure: Parvovirus infection (NN), drugs (esp cytotoxic) (NN), Fe deficiency (Microcytic Hypochromic) B12/folate deficiency (microcytic, Normochromic)
Anaemia due to bone marrow infiltration (NN)
Fe deficiency (Microcytic, Hypochromic)

57
Q

What is extramuedullary haemopoiesis?

A

haemophoisis outside of the bone marrow, usually associated with marrow metastasis or fibrous depositions.

58
Q

what is extra medullary haempoisis indicated by?

A

tear drop poikilocytes, NRBCs, Immature WBCs

59
Q

What is the treatment for ion deficient anaemia?

A 
Replenishment of ion stores:
    oral administration
    parenteral (injection)
Blood transfusion in severe cases
Rectify underlying causes (e.g. diet, malabsorption, bleeding)
60
Q

What are the causes or impairment of erythropoiesis?

A 
Fantom Anaemia
Damon Blackfan anaemia
   Present in early infancy
   Decrease in erythroid precursors
   Treat with steroids
   increased incidence of MDS and AML
61
Q

What are the causes of acquired haemolytic anaemia? (AUTOIMMUNE)

A 
polychromatic RBSs
microspherocytes
(NORMOCYTIC,NORMOCHROMIC)
red cell destruction is premature
increased reticulocytes
62
Q

What is microangiopathic?

A 
mechanical fragmentation, commonly associated with:
   mechanical heart valves
   DIC
   HUS/TTP
NORMOCYTIC/NORMOCHROMIC
63
Q

Give some examples of enzymopathies

A 
pyruvate kinase deficiency > haemolysis through failure of glycolytic pathway > inadequate ATP
Causes:
   Sputnik Cells
   Cannot regulate water retention
NORMOCYTIC NORMOCHROMIC
64
Q

Oxidation of Hb

A

G6PD deficiency
Hb oxidises to MetHb
Spleen removes chunks of MetHb -> KERATOCYTES
Challenge by oxidising agent results in significant haemolysis
NORMOCYTIC,NORMOCHROMIC

65
Q

what is the most significant Hb variant in heamoglobinopathy?

A

Beta chains

66
Q

What is Sickle cell disease (HbSS)?

A

HbSS is insoluble in deoxy state, forms long crystals distorting RBC

67
Q

What type of anaemia does sickle cell disease cause?

A

NORMOCYTIC NORMOCHROMIC - balance polymorphism

68
Q

give 2 examples of unstable Hbs

A

HbKoln and HbZurick

69
Q

what kind of anaemia does unstable Hbs cause?

A

haemolytic anaemia

70
Q

Give some causes of unstable HBs

A
  1. abnormality of harm pocket, so harm is not firmly bound and water can enter >metHb
  2. Inteference in binding of alpha and beta chains
  3. inteference with alpha chain structure
71
Q

What is the result of unstable Hb?

A

Oxidation of haem iron, which precipitates and damages the cell membrane.
precipitates are called Heinz bodies

72
Q

Describe haemoglobinopathy the Thalassemia syndrome

A

group of inherited RBC disorder characterised by reduced global chain synthesis.
Generally prevalent in population that evolved in warm humid areas.

73
Q

What is Alpha thalassemia?

A

Impaired ability to synthesise alpha global chains, therefore you have excess beta chains forming beta tetramers > HbH

74
Q

What kind of anaemia doe Alpha thalassemia cause?

A

mild Microcytic, hypochromic

75
Q

What is beta thalassemia?

A

impaired ability to synthesis beta global chains, as a result in HbB gene on chromosome 11. excess alpha chain bind to RBC membrane damaging it, ineffective erythropoiesis and reduced RBC survival

Clinical Haematology (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions