Normocytic anaemia Flashcards

1
Q

What is the MCV range for normocytic anaemia?

A

80-95

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2
Q

What are the 2 pathways for normocytic anaemia?

A

Haemolytic and non-haemolytic

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3
Q

What are haemolytic causes for normocytic anaemia?

A

Sickle cell
G6PD deficiency
Autoimmune Haemolytic Anemia
Hereditary spherocytosis

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4
Q

What are non-haemolytic causes for normocytic anaemia?

A

Aplastic
Chronic disease (CKD)
Pregnancy
Myelophthisic process

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5
Q

True or false: bone marrow is failing in haemolytic anaemia?

A

False

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6
Q

Where is sickle cell anaemia most common?

A

Africa

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7
Q

What positive property does sickle cell anaemia provide?

A

Anti-malaria

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8
Q

What is the affected genotype in sickle cell anemia?

A

HbS HbS

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9
Q

In sickle cell anaemia, the “GAG” becomes GTG on the _th codon of the beta globin

A

6th

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10
Q

The GTG codes for which amino acid (instead of glutamic acid by GAG)

A

valine

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11
Q

What does the substitution of valine for glutamic acid cause to happen?

A

Irreversible RBC sickling

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12
Q

In sickle cell anaemia, RBCs are more fragile and have decreased s_____ a___ so less efficient at delivering oxygen

A

surface area

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13
Q

Sickle cells are more r___ and less f_____ so can become trapped and block small blood vessels

A

rigid, flexible

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14
Q

There is a risk of s______ with sickle cells anaemia which means…

A

sequestration
accumulation particularly in the spleen, decreasing the lifespan of the cells

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15
Q

The altered shape of sickle cells makes them more susceptible to h_____, they can rupture and release haemoglobin into the bloodstream

A

haemolysis

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16
Q

What can haemolysis visibly cause

A

Jaundice

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17
Q

What are symptoms of sickle cell anaemia?

A

General anaemia and jaundice with complications like:
splenic sequestration (can cause it to shrink = auto splenectomy)
Vaso-occlusive crises, particularly in long bone vessels
Acute chest crisis (vaso-occlusion causing respiratory distress)

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18
Q

What factors can worsen the symptoms of sickle cell anaemia?

A

infections, cold, hypoxia, acidosis and dehydration

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19
Q

What other condition are individuals with sickle cell anaemia particularly susceptible to?

A

Infections like osteomyelitis from salmonella (due to lack of blood supply to bone leading to bacterial growth)

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20
Q

What investigations are there for sickle cell anaemia?

A

New-born screening (Gunthe heel prick test)

FBC and blood film (showing normocytic normochromic with increased reticulocytes, sickled RBCs and Howell Jolly bodies)

Haemoglobin electrophoresis (show 90% HbS)

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21
Q

What are Howell Jolly Bodies

A

Peripheral bodies within RBC representing DNA material

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22
Q

What is treatment for acute complicated attacks of sickle cell anaemia?

A

IV fluid
NSAIDS (analgesia)
Oxygen
Antibiotics if infection

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23
Q

What is long-term management of sickle cell anaemia?

A

Avoid precipitants (factors that worsen it eg the cold)
Hydroxycarbamide and folic acid supplements
Transfusion and iron chelation

Last resort = Bone marrow stem cell transplant

24
Q

What does “G6PDH” deficiency stand for?

A

Glucose-6-phosphate dehydrogenase

Involved in glutathione synthesis and protective against oxidative damage

25
Q

What is the cause of G6PDH deficiency?

A

X linked recessive enzymopathy

26
Q

What does glutathione protect against?

A

Reactive oxygen species like H2O2 (therefore protects against haemolysis)

27
Q

What are precipitating factors for G6PDH deficiency anaemia?

A

Nitrofurantoin (antibacterial)
Napthelene (found in moth balls and pesticides)
Antimalarials (like quinine)
Aspirin
Fava beans (contains glucosides that are metabolised to ROS)

28
Q

What are symptoms of an attack of G6PDH deficiency?

A

rapid anaemia and jaundice

29
Q

How is G6PDH diagnosed?

A

FBC and blood film (during attack will show normocytic normochromic with increased reticulocytes and Heinz bodies and bite cells

Low G6PDH levels

30
Q

What do Heinz bodies indicate?

A

Oxidative injury to RBC

31
Q

Bite cells are RBCs with irregular, “punched-out” membranes which result from removal of denatured ______ by macrophages in the spleen

A

haemoglobin

32
Q

How do you treat G6DPH deficiency anaemia?

A

Avoid precipitants
Blood transfusion when attacks

33
Q

What is hereditary spherocytosis?

A

Autosomal dominant membranopathy (RBCs become spherical shape and fragile)

34
Q

Where is hereditary spherocytosis most common?

A

Northern Europe and America

35
Q

Hereditary Spherocytosis is due to a deficiency in some of the structural membrane proteins such as s_____, making RBCs more spherical and rigid

A

spectrin

36
Q

The splenic m____ recognises the spherocytic cells as abnormal and target them for destruction

A

macrophages

37
Q

The increased destruction of spherocytic cells in the spleen leads to h______, contributing to release of haemoglobin into the blood stream and anaemia

A

haemolysis

38
Q

In spherocytic anaemia, the breakdown of haemoglobin results in the release of ______ contributing to jaundice

A

bilirubin

39
Q

The rigid spherocytic cells can also get stuck in the spleen, causing s______

A

splenomegaly (and risk of autosplenectomy)

40
Q

What are symptoms of hereditary spherocytic anaemia?

A

General anaemia
Neonatal jaundice
Splenomegaly
Gall stones (50%)

41
Q

Why can spherocytic anaemia cause gall stones?

A

The premature and excessive breakdown of the spherocytic RBCs leads to increased bilirubin production. Can overwhelm liver’s capacity to process and result in impaired bilirubin excretion with increased concentration in bile. The unconjugated bilirubin is less soluble in water so can precipitate to form stones.

Also breakdown of haemoglobin, leads to increased cholesterol levels contributing to cholesterol gallstones.

42
Q

What are investigations for hereditary spherocytic anaemia?

A

FBC and blood film (showing normocytic normochromic cells and increased reticulocytes, spherocytes)
Direct Coombs (-ve)
AHA (autoimmune haemolytic anaemia - +ve?)

43
Q

What is the direct Coombs test?

A

Detects antibodies or complement proteins on the surface of RBCs. Positive results may indicate autoimmune haemolytic anaemia (AHA).
May also be used in blood typing and compatibility testing.

44
Q

What is the treatment/ management for hereditary spherocytic anaemia?

A

Splenectomy (needs to be 6 years old otherwise risk of sepsis as spleen fights off encapsulated bacteria)

Folate supplements
Transfusions

45
Q

How would you treat a neonate with jaundice as a result of hereditary spherocytic anaemia?

A

Phototherapy, the light can break down the unconjugated bilirubin
(risk of kernicterus if untreated where bilirubin accumulates in Basal ganglia leading to CNS dysfunction and death).

46
Q

What are the 2 types of autoimmune haemolytic anaemia (AIHA)?

A

Warm and cold

47
Q

What are the features of warm antibody AIHA?

A

Antibodies most active at body temperature and typically involve IgG antibodies binding to the surface of RBCs leading to their premature destruction.
Most common.

48
Q

What are the features of cold antibody AIHA?

A

Antibodies more active at lower temperatures and the extremities. Typically involve IgM antibodies. Cold leads to clumping of RBCs and destruction.

49
Q

What testing is done to diagnose AIHA?

A

Direct Coombs +ve (agglutination of RBCs with Coombs reagent)

50
Q

True or false, the coombs result is positive in hereditary spherocytic anaemia?

A

False, it is negative

51
Q

How can CKD cause non-haemolytic anaemia?

A

Damaged kidneys produce less erythropoietin so less stimulation for bone marrow to undergo erythropoiesis.

Cells are normocytic and normochromic but there can be increased or decreased reticulocytes

52
Q

What is pancytopenia?

A

A deficiency in all 3 cellular components of blood (RBCs, WBCs, platelets)

53
Q

What is aplastic anaemia?

A

A pancytopenia where bone marrow fails and stops making hemopoietic stem cells.

Not understood why it is acquired, idiopathic, could be linked to infection or be congenital.

54
Q

What are investigations for aplastic anaemia?

A

FBC show normocytic anaemia with decreased reticulocytes
Bone marrow biopsy shows hypocellularity
Can give broad spectrum antibiotics due to high risk of infection
Bone marrow transplant

55
Q

What is another cause of non-haemolytic anaemia other than CKD and aplastic anaemia?

A

Malignancy

56
Q

How can a malignancy cause non haemolytic anaemia?

A

Malignancies can infiltrate the bone marrow and disrupt the normal haematopoiesis, reducing the production of RBCs or displacing hte haematopoietic cells (myelophthisis)

Could also affect hormonal regulation including erythropoietin (EPO) levels or impair the kidneys to produce EPO.

Chronic bleeding due to a malignancy (eg GI cancer) can lead to iron deficiency of malabsorption of B12.

Also cancer treatments can suppress the bone marrow’s ability to produce cells.