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1. Haematology > Microcytic Anaemia > Flashcards

Microcytic Anaemia Flashcards

1
Q

What is the Hb criteria for anaemia for males?

A

<130g/L

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2
Q

What is the Hb criteria for anaemia for females?

A

<120g/L

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3
Q

When are Hb and Hct not good markers for anaemia?

A

when its not in steady state eg rapid blood loss

or in haemodilution

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4
Q

How is the response to anaemia measured?

A

reticulocyte count

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5
Q

What leads to a microcytic anaemia?

A

shortage in globin or heme (porphyrin ring and iron)

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6
Q

What is microcytic anaemia?

A

low MCV

hypochromatic

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7
Q

What causes a microcytic anaemia?

A

Heme deficiency - iron deficiency + anaemia of chronic disease
porphyrin ring synthesis - lead poisoning, pyridoxine responsive anaemia, sideroblastic anaemia
globin deficiency - thalassaemia

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8
Q

What is the role of iron?

A

oxygen transport - Hb and myoglobin

electron transport - mitochondrial production of ATP

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9
Q

What is iron stored in in the liver?

A

Ferritin

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10
Q

What is iron stored in in the body?

A

haemaglobin

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11
Q

What is circulating iron bound to?

A

transferrin

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12
Q

What does transferrin pass iron onto?

A

bone marrow macrophages that feed it to red cell precursors

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13
Q

What are the properties of transferrin?

A

protein with two binding sites for iron atoms
transports iron from donor tissues (macrophages, hepatocytes + intestinal cells) to tissues expressing transferrin receptors

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14
Q

How do you measure iron supply?

A

% saturation of transferrin with iron

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15
Q

How do you measure iron storage?

A

serum ferritin

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16
Q

How does ferritin react to acute inflammation/disease?

A

increases so binds lots of iron so the bacteria cannot feed on the iron

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17
Q

What are the causes of iron deficiency anaemia?

A

menorrhagia
GI - tumours, ulcers, NSAIDs
haematuria
coeliac disease - not absorbing enough from proximal small bowel

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18
Q

How many mg a day do you need iron intake wise?

A

1mg a day

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19
Q

What ml of blood and mg of iron do you lose on average each menstrual cycle?

A

30-40ml

15-20mg iron

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20
Q

What are the consequences of iron deficiency?

A

epithelial changes - dry skin and koilonychia of nails
microcytic anaemia
iron deficient erythropoesis
exhaustion of iron stores

21
Q

What are the different variants of Hb?

A

HbA
HbF
HbA2 - 2 aplha 2 delta

22
Q

What chromosome are alpha genes found? How many per chromosome?

A

chromosome 16

2 genes per chromosome 4 per cell

23
Q

What chromosome are beta genes found? How many per chromosome?

A

chromosome 11

1 gene per chromosome 2 per cell

24
Q

What are haemoglobinopathies?

A

abnormal globin chain synthesis caused by autosomal recessive disorders leading to microcytic anaemias

25
Q

What are the types of haemoglobinopathies?

A

thalassaemias

structural haemoglobin variants

26
Q

What are the two types of thalassaemias?

A

alpha and beta

27
Q

What is an alpha thalassaemia?

A

will result from deletion of 1 alpha or both

i.e. can be reduced chain production or absent chain production

28
Q

What are the 3 types of alpha thalassaemias?

A 
alpha thalassaemia trait (-a,aa)
HbH disease (--,-a)
Hydrops foetalis (--,--)
29
Q

How does Alpha thalassaemia trait present?

A

asymptomatic

microcytic hypochromatic cells with a mild anaemia

30
Q

What treatment is required for Alpha thalassaemia trait?

A

NONE

31
Q

How is Alpha thalassaemia trait distinguished from iron deficiency anaemia?

A

ferritin

32
Q

How does HbH disease present?

A

more severe
anaemia with very low MCV and MCH
jaundice and splenomegaly

33
Q

Where is HbH disease common?

A

SE asia

34
Q

How is HbH disease treated?

A

splenectomy
transfusions
folic acid 1mg

35
Q

What is Hydrops foetalis?

A

no alpha chain to bind to so tetramers of Hb Barts and HbH produced
most severe form

36
Q

How does Hydrops foetalis present?

A 
profound anaemia
cardiac failure
skeletal and CUS abnormalities
growth retardation
severe hepatosplenomegaly
BUT NORMALLY PICKED UP ON ANTENATAL SCREENING AND DIE IN UTERO
37
Q

What are the types of beta thalassaemia?

A

b thalassaemia trait (B+/B) or (B0/B)
b thalassaemia intermedia (B+/B+) or (B0/B+)
b thalassaemia major (B0/B0)

38
Q

What causes beta thalassaemia?

A

disorder of B chain synthesis, usually caused by point mutations
can be reduced (B+) or absent (B0) B depending on the mutation

39
Q

What type of Hb does beta thalassaemia affect?

A

adult Hb - 2 alpha 2 beta

40
Q

How does b thalassaemia trait present?

A

asymptomatic

mild/no anaemia

41
Q

How is b thalassaemia trait tested for?

A

raised HbA2 in blood

42
Q

How does b thalassaemia intermedia present?

A

moderate anaemia - jaundice and splenomegaly

43
Q

How is b thalassaemia intermedia treated?

A

transfusion ocassionaly

44
Q

How does b thalassaemia major present?

A

severe anaemia
6-24 months as HbF falls
failure to thrive, pallor, hepatomegaly, organ changes and skeletal changes - all due to extramedullary haematopoesis

45
Q

How is b thalassaemia major treated?

A

lifelong transfusion

46
Q

What is the main problem with b thalassaemia major?

A

iron overload from transfusion

47
Q

How does iron overload present?

A

endocrine dysfunction - diabetes, osteoporosis, cardiac disease, liver disease

48
Q

How do you treat iron overload?

A

iron chelating drugs - desferioxamine

1. Haematology (24 decks)

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