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Haematology > Haemolytic anaemias > Flashcards

Haemolytic anaemias Flashcards

1
Q

Haemolytic anaemias are either inherited or acquired. Name the categories and subcategories of classification under these 2 broad headings.

A 
Inherited 
- abnormal globin synthesis 
-Enzymopathies 
- RBC membrane disorders 
Acquired 
- Immune
     *Alloimmune
     *Drug induced 
     *Autoimmune 
          -->Warm 
          -->Cold
-non-immune
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2
Q

Classify completely the type of anaemia thalasaemia falls under (by pathology as well as MCV)

A

Inherited abnormal globin synthesis hypochromic microcytic anaemia.

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3
Q

Classify the different types of alpha thalasaemia and what they result in

A
  • loss of 4 alpha genes = intrauterine death and hydrops faetalis
  • loss of 3 genes= results in HbH disease
  • loss of 1/2 genes= alpha thalaseamia trait, hypochromic microcytosis and a raised reticulocyte count.
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4
Q

How to diagnose alpha thalasaemia

A

alpha globin chain sequencing

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5
Q

What facies does Beta thalasaemia result in

A

maxillary expansion and skull vault expansion

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6
Q

Describe the characteristics of beta thalasaemia major

A
  • jaundice
  • severe hypochromic microcytic anaemia
  • hepatosplenomegaly
  • bone abnormalities
  • poor growth
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7
Q

How do you treat beta thalasaemia major

A
  • Blood transfusions

- lifelong iron chelation

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8
Q

Describe the characteristics of the beta thalasaemia trait

A
  • mild hypochromic microcytic anaemia

- asmptomatic

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9
Q

How do you diagnose beta thalasaemia major

A
  • high reticulocyte count
  • basophilic stippling
  • target cells
  • an increase in HbF and a decrease in HbA
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10
Q

What is the difference between the Hb types seen in thalasaemia (beta) trait and thalasaemia major

A

In thalasaemia major= increase in HbF and decrease in HbA
In thalasaemia trait= increase in HbA2 and a decrease in HbA
note: Both will have an increased reticulocyte count

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11
Q

What causes sickle cell anaemia?

A

An inherited recessive trait which results in the amino acid glycine been replaced by valine at position 6 of the beta globin gene.

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12
Q

What gives the cells the sickle shaped appearence?

A

Polymerisation of the abnormal globin chain when it is deoxygenated leading to insoluble aggregates which deform the RBC.

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13
Q

What does the sickle shape lead to?

A
  • splenic trapping
  • vascular occlusion
  • leg ulcers
  • haemolysis
  • pulmonary hypertension
  • CRF due to glomerular damage
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14
Q

How do you diagnose sickle cell anaemia?

A
  • Sickle cell prep
  • Sickle cells on PB smear
  • Hb electrophoresis
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15
Q

How do you treat sickle cell anaemia?

A
  • Immunise against infections
  • Treat infections early
  • Give folate
  • Blood transfusions
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16
Q

Fully classify the type of anaemia a G6PD deficiency results in.

A

Haemolytic enzymopathy (bite cells on peripheral blood smear)

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17
Q

How does a G6PD deficiency cause problems?

A

There is oxidative damage to the red blood cells (metHb and Heinz-Bodies form) this results in haemolysis. There is a decrease in NADPH synthesis resulting in an inability to maintain GSH levels.

18
Q

How do you diagnose G6PD deficiency?

A

Simple- check for the enzyme levels in the blood.

19
Q

Why does the way of diagnosing G6PD pose a problem in acute episodes.

A

The enzyme levels may be increased in the reticulocytes leading to a falsely normal level showing up.

20
Q

How do we treat G6PD deficiency?

A
  • Treat infections

- avoid axidant drugs

21
Q

What can aggravate the low grade chronic anaemia of G6PD deficiency and result in an acute episode?

A
  • fava bean exposure
  • infections
  • oxidative drugs
22
Q

An RBC membrane defect of the horizontal axis will result in?

A

Hereditary eliptocytosis

23
Q

An RBC membrane defect of the vertical axis will result in?

A

Hereditary spherocytosis

24
Q

What is the commonest non-immune haemolytic anaemia in caucasions and how is it transmitted?

A

Hereditary spherocytosis. It is autosomal dominant and therefore congenital.

25
Q

Why is there glucose depletion in H.spherocytosis

A

The spherical cells result in a decreased deformability which results in glucose trapping and metabolic stress.

26
Q

How do we treat H.spherocytosis?

A
  • splenectomy if the anaemia is severe

- folate supplements

27
Q

How do we diagnose H.spherocytosis?

A
  • Take a fam history
  • PB smear
  • Osmotic fragility test
  • Coombs negative
28
Q

How does warm AI haemolytic anaemia occur (WAIHA)?

A
  • secondary to an underlying infection which results in polycolonal B cell activation and and the synthesis of IgG autoantibodies as well as complement activation resulting in extravascular haemolysis.
29
Q

How do you diagnose WAIHA?

A

By a +DAT (COOMBS test- for IgG or IgG+complement)

30
Q

How do you treat WAIHA?

A
  • folate
  • splenectomy
  • steroids
31
Q

What is CAIHA caused by?

A

Can be:

  • idiopathic
  • secondary to lymphoproliferative disorder or infection.
32
Q

How do you treat CAIHA?

A
  • avoid cold

- treat underlying infection.

33
Q

Is CAIHA microcytic or macrocytic?

A

Macrocytic

34
Q

How does CAIHA present?

A
  • mild anaemia

- jaundice with acrocyanosis

35
Q

What makes cross matching difficult in CAIHA?

A
  • haemaglutins
36
Q

What can acquired non Immune haemolytic anaemias be caused by?

A
  • infections
  • fragmentation haemolysis
  • chemical or physical agents
37
Q

What are two microbes which can result in non immune haemolytic anaemia?

A
  • C.perfringens

- plasmodium

38
Q

In an infection with C.perfringens what are you likely to see on a PB smear?

A

Ghost cells and spherocytes

39
Q

Malaria can result in a condition known as blackwater fever- describe its presentation.

A
  • IV haemolysis
  • haemaglobinuria
  • oliguric RF
40
Q

How can oxidative agents result in anaemia?

A
  • haemoglobin is oxidised to methaemoglobin
  • Heinz bodies are formed
  • oxidation of RBC membrane results in IV haemolysis
41
Q

How can thermal injuries (burns) result in anaemia?

A

They result in IV haemolysis and haemoglobinuria.

Haematology (3 decks)

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