Anaemia

Question 1

Hypochromic Microcytic blood film is seen in? (5)

Answer 1

Iron deficiency
Sideroblastic anaemias
Lead poisoning
Thalassemia traits
Anaemia of chronic disease

Question 2

What type of anaemia is seen in folate deficiency?

Answer 2

Macrocytic anaemia

Question 3

Most common type of anaemia in children?

Answer 3

Iron deficiency anaemia (microcytic)

Question 4

Name 3 causes of a normocytic anaemia

Answer 4

Blood loss
Mixed nutritional
TEC

Question 5

Most 2 common causes of microcytic anaemia?

Answer 5

Iron deficiency

Thalassemia/spherocytosis

Question 6

Physiological anaemia occurs when in;
Term babies?
Preterm babies?

Answer 6

Term babies: 8-12wks

Preterm babies: 6-8wks

Question 7

What decreases Iron absorption?

Answer 7

calcium (don’t take with BF cereal)

Question 8

What increases iron absorption?

Answer 8

increased acid (drink orange juice 30mins prior)

Question 9

What is hepcidin?

Answer 9

hormone which regulates iron metabolism

Question 10

What are the stages of iron deficiency?

Answer 10

1. Iron depletion (dec ferritin, N Hb + indices)
2. Iron deficiency (low ferritin, low indices, N Hb)
3. Iron def anaemia (low Hb)

Question 11

Response after commencing oral iron?

Answer 11

2-5 days: increased retics

7-10 days: increased Hb

Question 12

Response after commencing oral iron?

Answer 12

2-5 days: increased retics

7-10 days: increased Hb

Question 13

Clinical findings with B12 deficiency?

Answer 13

Megaloblastic anaemia
Neurological Cx (posterior columns, pyramidal tracts, peripheral neuropathy, depression, dementia, seizures)

Question 14

Define hemolysis?

Answer 14

More rapid breakdown of RBC

Question 15

Features of haemolytic uraemic syndrome?

Answer 15

Microangiopathic hemolytic anaemia
Fever
Renal impairment
Thrombocytopenia

Question 16

What drugs can cause Thrombotic Thrombocytopenic Purpura (TTP)?

Answer 16

Chemo
Clopidogrel
Cyclosporin
Quinine

Question 17

Mortality with TTP?

Answer 17

50%

Question 18

Pathological process of hereditary spherocytosis?

Answer 18

Deficiency in a protein responsible for cytoskeleton of RBC

Question 19

Inheritance of hereditary spherocytosis?

Answer 19

75% AD

25% spontaneous mutations

Question 20

What should you exclude if a child presents with gallstones?

Answer 20

Hereditary spherocytosis

Question 21

G6PD inheritance?

Answer 21

X-linked

Question 22

Age by which 90% children with sickle cell disease have functional asplenia?

Answer 22

6yrs

Question 23

Complications of sickle cell disease?

Answer 23

Vascular occlusion (stroke, dactylitis, pripism)
Splenic sequestration
Hyper-hemolysis
Infections
Aplastic crisis

Question 24

Pathophysiology of sickle cell disease?

Answer 24

Molecular (consequently structural) defect in B-globin chains, meaning cells polymerise on deoxygenation (sickle)

Question 25

Leading cause of death in sickle cell disease?

Answer 25

Infection

Question 26

Features of thalassemic facies?

Answer 26

Frontal bossing Maxilla hyperplasia Flat nasal bridge

Question 27

Alpha-thal trait, how many alpha genes are deleted?

Answer 27

2

Question 28

HbH disease means how many alpha genes are deleted?

Answer 28

3

Question 29

What does alpha-thalassemia major cause?

Answer 29

Death in utero (no HbF formation)

Question 30

What is Constant spring disease?

Answer 30

Non-deletional alpha-gene mutation | causes abnormal Hb and more severe anaemia than deletion forms of alpha-thal

Question 31

Warm haemolytic anaemia is caused by?

Answer 31

IgG (>37 degrees)

Question 32

Cold haemolytic anaemia is caused by?

Answer 32

IgM (<37 degrees)

Question 33

What infection is associated with B-thalassemia (and why)?

Answer 33

Yersinia (iron overload 2 transfusions)

Question 34

What congenital anomalies are associated with Fanconi's anaemia?

Answer 34

``` Radial hypoplasia Abnormal thumbs Hyperpigmented (cafe au lait) Pancytopenia Hypogonadism ```