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ANAEMIA Flashcards

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1
Q

Anaemia is classed as low red blood cells what the 2 main markers?

A

Haemoglobin - Conc. measured by light absorbed (F <120, M<130)
Haematocrit (hct) - % of RBCs in total blood

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2
Q

Why are hct and Hb not good markers in situations of:

a) acute haemorrhage
b) Large volume resuss

A

a) blood loss will give FALSE POSITIVE (for anaemia). Proportional blood loss means sample hct and Hb are constant. Even though TOTAL RBCs are LOW.
b) HAemodilution decreases the % of the sample that is RBCs even though their level may be fine. FALSE NEGATIVE (for anaemia)

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3
Q

How is anaemia sensed and responded to by the body?

A

Kidney senses hypoxia and releases EPO. In response bone marrow undergoes increased reticulocytosis.

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4
Q

Anaemia and no increase in reticulocytosis indicates?

A

Impaired marrow response.

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5
Q

Reticulocytes stain deeper (blue/purple) than erythrocytes because they contain a nucleus T/F?

A

F. Have just gotten rid of their nucleus but have residual RNA.

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6
Q

When is a reticulocyte classed as an erythrocyte

A

When it loses its RNA around 7 days in circulation.

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7
Q

How are reticulocytes different to erythrocytes on blood film?

A

They are
- polychromatic
Darker
Stain DEEPER

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8
Q

General presentation of anaemia?

A

Exertional SOB, dizzy/faint, worsening angina. Fatigue, headache

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9
Q

Why does impaired haemoglobinisation cause microcytic anaemia?

A

Hb is synthesised in the cytoplasm => problem with its production –>defective cytoplasmic maturation.

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10
Q

Most common cause of microcytic hypochromic anaemia?

A

Iron deficiency anaemia.

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11
Q

Which part of haemoglobin is iron responsible for the production of?

A

HAEM

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12
Q

What is the cause of microcytic anaemia due to the impaired production of GLOBIN?

A

Thalassemia.

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13
Q

In acute scenarios when Hb is <70 a transfusion is indicated. Regular transfusions are indicated in the management of which anaemia cause?

A

Thalassemia. (HbH and Beta thalassemia major and minor)

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14
Q

What is the mechanism by which impaired nuclear division causes macrocytic anaemia?

A

Cells fail to become smaller (macrocytic) and so are more prone to apoptosis. LESS cells which are BIG.

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15
Q

Blood film of megaloblastic macrocytic anaemia?

A

Hypersegmented neutrophills, oval macrocytes. Howel jolly bodies

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16
Q

2 causes of megaloblastic anaemia?

A
  1. Vitamin B12 and folate deficiency

2. Drug induced toxicity

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17
Q

How do causes of non-megaloblastic anaemia cause big cells?

A

Altered red cell membrane.

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18
Q

Non-megaloblastic macrocytosis always presents with anaemia? T/F

A

F. Can present with or without. Alchohol, hypothyroidism, liver disease, pregnancy may present with isolated macrocytosis.

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19
Q

Cause of non-megaloblastic macrocytosis that will always cause anaemia?

A

Bone marrow failure.

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20
Q

2 causes of false macrocytoisis?

A

Reticulocytosis.

Cold agglutins.

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21
Q

what is the difference between anaemia of chronic disease and iron deficiency anaemia?

A

Anaemia of chronic disease has INCREASED FERRITIN LEVEL. Secondary to inflammatory process. (ferritin = also a reactive protein) and DECREASED TIBC.

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22
Q

Why does anaemia of chronic disease cause decreased plasma iron and => microcytic hypochromic anaemia?

A

Chronic disease release of cytokines stimulates liver protein hepcidin which inhibits the exit of iron from cells (enterocytes, bone marrow cells, macrophages, liver).

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23
Q

What should be considered in all microcytic hypochromic anaaemias NOT responding to treatment?

A

Sideroblastic anaemia. Rare x-linked condition.

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24
Q

Sideroblastic anaemia can happen in response to?

A

TB treatment (RIPE antibiotics) or Chemo

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25
Siderolastic anaemia causes HIGH serum iron how?
HIGH IRON. Problem with porphyrin RING formation => cannot bind to iron and make haem. Relative excess iron which is then absorbed and deposited in other tissues.
26
Treatment of sideroblastic anaemia?
REGULAR transfusions.
27
Which form of beta thalassaemia is most likely to be misdiagnosed as iron deficiency anaemia?
beta T intermedia. As Beta T trait is usually clinically asymptomatic.
28
Severe anaemia and failure to thrive in a 6 month old baby with a family history of haemoglobinopathies. What is the most likely cause.
BETA thalassaemia MAJOR. Alpha T does not present at a young age unless its the most severe form Hb Barts, but in this case children will rarely live past birth. Beta T major has severe clinical manifestations and presents in 1st year of life.
29
Specific sign for HbH alpha thallasaemia seen on blood film?
Heinz bodies. Indicate presence of HbH tetramers.
30
Management of clinically apparant thalassemia?
Folic acid Transfusion (intermittent or lifelong) +/-splenectomy
31
WHat is responsible for the mortality of thalassemia and what is done to prevent this?
Iron overload for those receiving regular transfusions => given iron chelators (defiriprone)
32
Primary haemoglobin affected in sickle cell disease?
HbA (adult Hb)
33
Sickling disorders involve a point mutation of ___ gene on chromosome __ ?
``` HbB gene (haemaglobin beta gene) Chromosome 11 ```
34
what is the effect of this mutation of the beta gene?
beta chain produced is structurally abnormal which results in and abnnormal HbS haemaglobin.
35
How many copies of this mutation are needed to result in sickle cell ANAEMIA?
2. Autosomal recessive. | If its just one its called a trait.
36
Sickle cell trait will cause no problems unless precipitated by?
Dehydration or in high altitude. Make RBCs sickle.
37
Why are sickle cells cresent shaped?
Abnormal Hb HbS polymerises and distorts => crescent shaped RBC with rough membrane.
38
How is sickle cell diagnosed?
Electrophoresis
39
Why does sickle cell cause anaemia?
Abnormal RBCs --> haemolysis (premature destruction) and chronic haemolysis (from repeated sickling)--> reduced RBC life span.
40
Why does organomegaly occur in sickle cell anaemia?
Chronic anaemia --> Extramedullary haemapoesis. Also splenic sequstration occurs (blood cells get trapped in spleen).
41
Why can some sickle patients present with dactylitis (when young), bone pain (later) and stroke?
VASO OCCLUSION. Sickle cells clog vessels. Causes pain and inflammation
42
When vasoocclusions affect pulmonary or cardiovascular systems it is termed...?
SICKLE CRISIS
43
Treatment of sickle crisis?
Red cell EXCHANGE transfusions. + opiods and fluid resuss.
44
Why are sickle cell patients offered prophylactic antibiotics and vaccinations?
Immunocomprimised. Vasoocclusions cause splenic infarcts + sequestration => spleen has reduced ability to clear macrophages that have picked up infection.
45
Which myelosuppressive agent is used to reduce future incidence of painful sickle episodes?
Hydroxycarbamide.
46
General management of sickle cell?
``` Folic acid Pain relief (opioids) Fluids and rest Antibiotics at signs of infection Regular exchange transfusions (in events of crisis or may need regularly to prevent stroke) ```
47
What is the mechanism of haemolytic anaemia?
Decompensated haemolysis - Premature RBC destruction
48
What is the bone marrow response to haemolysis?
Reticulocytosis and erythroid hyperplasia
49
Why are bony deformities be a consequence of chronic haemolytic anaemia?
Chronic erythroid hyperplasia (bone marrow compensation)
50
Presence of schistocyes (fragmented RBCs)indicates ____ haemolysis?
Intravascular
51
Clinical features of extravascular haemolysis?
Jaundice/gallstones. Excess uncongugated bilirubun.
52
Why is intravascular haemolysis more serious?
It causes an excess of ABNORMAL products.
53
Why do haptoglobins decrease in intravascular haemolysis?
They pick up free Hb
54
Test for immune haemolytic anaemias?
Coombs test - +ve if immune
55
Which haemolytic anaemia is caused by ABO transfusion reaction and HAemolytic disease of the newborn?
Alloimmune - bodies antibodies against non-self antigens
56
HOT/COLD autoimmune haemolytic anaemia is associated with leukemia and lymphoma and falsely presents with macrocytosis on blood film?
COLD
57
What is the mechansim of MAHA Microangiopathic Haemolytic Anaemia?
Mechanical stress --> intravascular haemolysis
58
Bite cells and heinz bodies associated with?
G6DP deficiency
59
Heinz cells can also be a sign of which thalassaemia?
HbH alpha thalassaemia
60
20 y/o mild intermittent jaundice, father had splenectomy?
Heridatory spherocytosis
61
Heridatory spherocytosis is autosomal?
DOMI~NANT
62
Management for HS?
Folic Acid. To counteract chronic extravascular hamolytic anaemia. Splenectomy if young and growth affected.

HAEMATOLOGY (3 decks)

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