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Clinical Haematology > RBCs, Anaemia and Haemaglobinopathy > Flashcards

RBCs, Anaemia and Haemaglobinopathy Flashcards

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1
Q

What is the average size of a red blood cell?

A

7um

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2
Q

What shape do the RBCs take?

A

Biconcave discs

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3
Q

Blood pH is between ____ - ______

A

Blood pH is between 7.35-7.45

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4
Q

Acidosis is a clinical condition where blood pH is below ____

A

7.35

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5
Q

The clinical symptoms of Acidosis are…

A

Headaches, lethargy, breathlessness

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6
Q

Some transmembrane proteins of the RBC lipid bilayer are…

A

Glycophorin C
Ankyrin
Spectrin
Tropomyosin

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7
Q

The role of Glycophorin A is

A

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

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8
Q

The role of band 3 protein is

A

anion transport, esp chloride and bicarbonate

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9
Q

The role of glycophorin C is

A

Regulating cell shape, membrane deformability, membrane mechanical stability

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10
Q

Ankyrin..

A

links lipid bilayer to spectrin

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11
Q

Spectrin is responsible for the

A

maintenance of biconcave discs, they are the most abundant protein

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12
Q

RBCs metabolic activity is

A

Anaerobic glycolysis

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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

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14
Q

Dehydration of RBC leads to _____ cells

A

Dehydration of RBS leads to SPUTNIK cells

MetHB cannot carry O2

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15
Q

The process of RBC production is called?

A

Erythropoiesis

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16
Q

In the Adult where does RBC production occur?

A

Bone marrow of sternum, pelvis and long bones

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17
Q

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

A

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

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18
Q

what is the role of Erythropoietin?

A

Erythropoietin acts upon a committed ERYTHROID precursor to increase cell division

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19
Q

In renal hypoxia is erythropoietin production increase or decrease?

A

Increase

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20
Q

Immature RBC are nucleated True or False?

A

True

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21
Q

What is the lifespan of a RBC?

A

120 days

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22
Q

an increased reticulocyte count means..

A

an increased rate of RBC production

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23
Q

What are senescent RBC killed by?

A

Macrophages

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24
Q

Where are senescent RBC killed?

A

In the Reticule endothelial system = SPLEEN

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25
Macrophages line the ______ ______ in the spleen
SPLENIC CORDS
26
The white pulp is the ..
primary lymphoid section
27
what causes hastened destruction of RBC by splenic macrophages int he red pulp?
loss of membrane pliability or Ab coating
28
List the particle removal stages:
1. Nuclear material (howell-jolly bodies) 2. Cytoplasmic organelles 3. Sideritic granules 4. Oxidised Hb (MetHb)
29
what is the weight of Hb in kDa?
68kDa
30
What is the structure of Adult Hb? (HbA)
α2 β2
31
What is the structure of HbA2 Hb?
α2δ2
32
What is the structure of fatal Hb? (HbF)
α2γ2
33
HbF has a _____ affinity for O2 than maternal HB, in order to ______ oxygen
HbF has a HIGHER affinity for O2 than maternal HB, in order to RECIEVE oxygen
34
have a ____ O2 affinity where pO2 is _____, but a _____ affinity where pO2 is ____
Have a HIGH O2 affinity where pO2 is HIGH, but a LOW O2 affinity where pO2 is LOW
35
2,3DPG binds preferentially to what state Hb?
deoxyHb, thus reducing O2 affinity
36
What effect does acidosis have on O2 affinity?
reduces affinity, otherwise known as the BOHR EFFECT
37
What causes the reduced affinity of HbF?
less active 2,3DPG
38
What is the problem with free global chains?
they are toxic to the kidneys, scavenge nitrous oxide, leading to vasoconstriction and hypertension
39
List some advantages of Artificial RBCs
``` >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
What is the definition of Anaemia?
Reduced concentration of Hb in the blood
41
What are the symptoms of anaemia?
``` Hb falls below 90-100g/L slow onset SOB weakness pallor lethargy palpitations headaches heart failure and confusion ```
42
What are some clinical signs of anaemia?
``` Concave nails Jaundice Leg ulcers Bone deformations Recurrent infection and/or bruising ```
43
What is the normal Mean cell Volume? (MCV)
80-101fl
44
What is the normal mean cell Hb (MCH) =Hb/RBC
27-34pg
45
What is the normal Mean Cell Corpuscular Hb Concentration (MCHC)? =Hb/HcT
300-350g/L
46
Classification of anaemia is by?
Impaired production compared with increased RBC loss/destruction, more usually by visual categorisation
47
Normocytic, normochromic anaemia is when
RBC are of normal volume (MCV) contain normal amount of Hb at the right conc (MCH,MCHC) RBC count is reduced
48
Give some examples of cases where NN anaemia can occur?
cute bleed, haemolysis, bone marrow failure, renal failure
49
Microcytic, Hypochromic anaemia is when
RBCs are reduced in volume, | Recused Hb at a lower concentration than normal, (MCV,MCH,MCHC)
50
what are some causes of Microcytic Hypochromic anaemia?
Fe deficiency Thalassemia Anaemia of Chronic disorder
51
Microcytic, Normochromic Anaemia is when
RBC count is low, RBS are increased in volume (MCV) Hb is at normal concentrations (MCH increased, MCHC normal)
52
Give some examples of where microcytic, normochromic anaemia can occur>
B12 or folate deficiency
53
List the clinical causes of anaemia
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
Cytoskeletal RBC disorders include:
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
How would you treat hereditary spherocytosis?
removal of the spleen | Prophalytic AB treatment after surgery
56
What are the causes of acquired impairment of erythropoiesis?
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
What is extramuedullary haemopoiesis?
haemophoisis outside of the bone marrow, usually associated with marrow metastasis or fibrous depositions.
58
what is extra medullary haempoisis indicated by?
tear drop poikilocytes, NRBCs, Immature WBCs
59
What is the treatment for ion deficient anaemia?
``` Replenishment of ion stores: oral administration parenteral (injection) Blood transfusion in severe cases Rectify underlying causes (e.g. diet, malabsorption, bleeding) ```
60
What are the causes or impairment of erythropoiesis?
``` Fantom Anaemia Damon Blackfan anaemia Present in early infancy Decrease in erythroid precursors Treat with steroids increased incidence of MDS and AML ```
61
What are the causes of acquired haemolytic anaemia? (AUTOIMMUNE)
``` polychromatic RBSs microspherocytes (NORMOCYTIC,NORMOCHROMIC) red cell destruction is premature increased reticulocytes ```
62
What is microangiopathic?
``` mechanical fragmentation, commonly associated with: mechanical heart valves DIC HUS/TTP NORMOCYTIC/NORMOCHROMIC ```
63
Give some examples of enzymopathies
``` pyruvate kinase deficiency > haemolysis through failure of glycolytic pathway > inadequate ATP Causes: Sputnik Cells Cannot regulate water retention NORMOCYTIC NORMOCHROMIC ```
64
Oxidation of Hb
G6PD deficiency Hb oxidises to MetHb Spleen removes chunks of MetHb -> KERATOCYTES Challenge by oxidising agent results in significant haemolysis NORMOCYTIC,NORMOCHROMIC
65
what is the most significant Hb variant in heamoglobinopathy?
Beta chains
66
What is Sickle cell disease (HbSS)?
HbSS is insoluble in deoxy state, forms long crystals distorting RBC
67
What type of anaemia does sickle cell disease cause?
NORMOCYTIC NORMOCHROMIC - balance polymorphism
68
give 2 examples of unstable Hbs
HbKoln and HbZurick
69
what kind of anaemia does unstable Hbs cause?
haemolytic anaemia
70
Give some causes of unstable HBs
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
What is the result of unstable Hb?
Oxidation of haem iron, which precipitates and damages the cell membrane. precipitates are called Heinz bodies
72
Describe haemoglobinopathy the Thalassemia syndrome
group of inherited RBC disorder characterised by reduced global chain synthesis. Generally prevalent in population that evolved in warm humid areas.
73
What is Alpha thalassemia?
Impaired ability to synthesise alpha global chains, therefore you have excess beta chains forming beta tetramers > HbH
74
What kind of anaemia doe Alpha thalassemia cause?
mild Microcytic, hypochromic
75
What is beta thalassemia?
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)

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