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MD2 Foundation > Thalassaemia, Sickle Cell and Anaemia > Flashcards

Thalassaemia, Sickle Cell and Anaemia Flashcards

1
Q

HbA=
HbF=
HbA2=

A 
HbA= a2b2 adult Hb
HbF= a2y2 fetal Hb (switch at 3-4 months)
HbA2= a2d2, 2% of adult Hb
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2
Q

type of anaemia produced by thalassaemia

A

microcytic hypochromic

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3
Q

how to differentiate between iron deficiency and thalassaemia anemia

A

IRON: pencil cells, more anisocytosis and anisopoikilocytosis. elevated RDW. reduced RCC
THALASSAEMIA BETA MINOR: RDW normal, RCC normal, minor elevation of HbA2.

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4
Q

what is RDW?

A

indicates variability of size and shape of rbcs

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5
Q

raised HbA2 in presence of hypochromic microcytic anemia would indicate

A

beta thalassaemia minor

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6
Q

what is alpha thalassaemia major? what Hb is present?

A

lost all the alpha genes.

presence of y4 Hb Barts. leads to Barts hydrops fetalis

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7
Q

what is HbH

A

4 beta chains present in alpha thalassaemia

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8
Q

type of anemia caused by alpha thalassaemia minor

A

microcytic hypochromic

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9
Q

how do we diagnose alpha thalassaemia minor

A

its hard because HbH and Barts are often not elevated to detectable levels

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10
Q

where is sickle cell most prevalent?

A

subsaharan africa eg. nigeria. should screen for sickle cell

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11
Q

what happens to sickle cells as they pass through the microcirculation

A

they sickle up, become less deformable, increase blood viscosity and can affect oxygen deliver to brain kidneys luigns spleen. can get infarctions.

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12
Q

cause of chest syndrome in sickle cell

A

necrosis of lung parenchyma

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13
Q

cause of renal infarction in sickle cell

A

sickle cells get lodged at the bottom of loop of henle where oxygen is lowest

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14
Q

consequences of neonatal sickle cell disease

A

initially protected due to HbF but then get abnormal growth and bone developemnt, more vulnerable to viruses

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15
Q

treatment of sickle cell

A
  • raise HbF (hydroxyurea, butyrates)
  • reduce HbS
  • transfusion (iron overload? need chelator therapy)
  • manage complications
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16
Q

why do we check for sickle cell before general anaesthesia

A

grave consequences, very bad.

17
Q

problems with regular transfusions for sickle cell patients

A

iron overload requiring chelator therapy

can make antibodies against other rare blood groups making blood matching very difficult

18
Q

what is anisocytosis

A

variable size of rbcs

19
Q

what is anisopoikilocytosis

A

variable size and shape of rbcs

20
Q

3 mechanisms of anemia

A
  • loss
  • impaired production
  • increased destruction
21
Q

what is transferrin

A

transports iron around the body, absorbs iron

22
Q

what is ferritin

A

a storage protein of iron

also an acute phase protein

23
Q

what is hepcidin

A

regulator of iron metabolism. regulates circulating iron, iron availble for utilisation by bone marrow

24
Q

causes of excess iron

A 
intake
transfusions
inherited conditions (hemochromatosis)
25
Q

side effects of iron replacement therapy

A

tarry stool
nausea
loss of appetitie

26
Q

what is anaemia of chronic disease?

what type of anemia does it cause?

A

inflammatory environment increases the amount of hepcidin, this reduces the amount of iron available for utilisation by the bone marrow causing normocytic or microcytic anemia

27
Q

findings on iron deficiency anemia of ferritin and transferring?

A

low ferritin

elevated transferrin

28
Q

findings on chronic disease anemia of transferrin and ferritin

A

ferritin is normal

transferrin is low

29
Q

5 findings of hemolytic anemia

A
  • elevated lactate dehydrogenase
  • low haptoglobin
  • reticulocytosis
  • change in rbc morphology
  • unconjugated hyperbilirubinemia
30
Q

3 patterns of red cell morpholgy in haemolysis

A
  • spherocytosis (hereditary or warm immune hemolysis)
  • microangiopathy (fragmented red blood cell aka schistocyte)
  • oxidative (bite cells and blister cells)
31
Q

what pattern of morphology does G6PD produce in rbcs

A

oxidative with bite and blister cells

32
Q

how can we test if anemia is immune mediated?

A

direct coombs test to find anti-red cell antibody bound to rbcs

33
Q

what is a reticulocyte

A

cell before erythrocyte, have nucleic acid but no nucleus

34
Q

what is microangiopahty

A

fragmented red blood cells on film, schistocytes etc

35
Q

what is aplastic anemia

how do you differentiate from hemolytic?

A

marrow has stopped making rbcs,

hemolytic will have reticulocytosis

36
Q

what causes macrocytic anemia

A

folate or B12 deficiency

37
Q

what is pernicious anemia

A

autoimmune reactoin against parietal cells in the stomach leading to a loss of intrinsic factor which prevents vitamin B12 absorption, leading to anemia

MD2 Foundation (10 decks)

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