Haemolytic Anaemia

Question 1

What is the name given to the proteins in normal plasma which bind haemoglobin?

Answer 1

Haptoglobin

Question 2

Which anaemia is classified as an Acquired haemolytic anaemia but has Intrinsic defects?

Answer 2

Paroxysmal nocturnal haemoglobinuria ( PNH)

Question 3

What are the examples of Hereditary Haemolytic anaemia due to membrane defects?

Answer 3

1. Hereditary Spherocytosis
2. Hereditary elliptocytosis

Question 4

What are the examples of Hereditary Haemolytic anaemia due to Metabolism defects ?

Answer 4

1.G6PD deficiency

2. Pyruvate kinase deficiency

Question 5

What are the examples of Hereditary Haemolytic anaemia due to Haemoglobin defects?

Answer 5

Genetic abnormalities (Hb S, Hb C, unstable)

Question 6

What are the causes of Intravascular Haemolysis?

Answer 6

1. Mismatched blood transfusion (usually ABO)
2. G6PD deficiency with oxidant stress.
3. Red cell fragmentation syndromes.
4. Some severe autoimmune haemolytic anaemias.
5. Some drug‐ and infection‐induced haemolytic anaemias.
6. Paroxysmal nocturnal haemoglobinuria.
7. March haemoglobinuria.
8. Unstable haemoglobin.
9. G6PD, glucose‐6‐phosphate dehydrogenase

Question 7

Which haemolytic anaemia is the most common in Northern Europe?

Answer 7

Hereditary Spherocytosis

Question 8

What is the principal treatment for Spherocytosis?

Answer 8

Splenectomy

Question 9

What is the name of the oxidant chemical in Fava beans?

Answer 9

Divicine

Question 10

What is the composition for Adult Haemoglobin?

Answer 10

α2 ß2 or A2 (α2 δ2)

Question 11

What is the composition for Foetal Haemoglobin?

Answer 11

F (α2 γ 2)

Question 12

Which antibody causes Paroxysmal cold haemoglobinuria?

Answer 12

Donath–Landsteiner antibody

Question 13

What is Intravascular Haemolysis?

Answer 13

The breakdown of red cells within blood vessels)

Question 14

What are haemolytic anaemias?

Answer 14

Haemolytic anaemias are defined as anaemias that result from an increase in the rate of red cell destruction.

Question 15

What are the general clinical features of Haemolytic Anaemia?

Answer 15

• Pallor of the mucous membranes
• Mild fluctuating jaundice
• Splenomegaly

Question 16

What is Aplastic Crises?

Answer 16

Aplastic crises may occur, usually precipitated by infection with parvovirus, which ‘switches off’ erythropoiesis.

Question 17

What are the Lab findings of Aplastic Crises?

Answer 17

Increase in Anaemia & Drop In Reticulocyte count!!!

Question 18

What other pathology may cause an Aplastic Crises?

Answer 18

Folate deficiency !!! The bone marrow becomes megaloblastic.

Question 19

What are the General lab findings of Haemolytic Anaemia ( Features of Red Cell breakdown)?

Answer 19

Features of increased red cell breakdown :

(a) serum bilirubin raised, unconjugated and bound to
albumin;
(b) urine urobilinogen increased;
(c) serum haptoglobins ABSENT because the haptoglobins
become saturated with haemoglobin and the complex is removed by RE cells.

Question 20

What are the General lab findings of Haemolytic Anaemia ( Features of Increased Red Cell Production)?

Answer 20

(a) Reticulocytosis
(b) bone marrow erythroid hyperplasia – the normal marrow myeloid : erythroid ratio of 2 : 1 to 12 : 1 is reduced
to 1 : 1 or reversed.

Question 21

What are the General lab findings of Haemolytic Anaemia ( Features of Damaged Red Cells)?

Answer 21

(a) routine blood film morphology (e.g. microspherocytes, elliptocytes, fragments);
(b) flow cytometry after eosin-maleimide (EMA) staining; (c) specific enzyme, protein or DNA tests.

Question 22

What are the main laboratory features of Intravascular Haemolysis?

Answer 22

1. Haemoglobinaemia and haemoglobinuria.
2. Haemosiderinuria.
3. Methaemalbuminaemia (detected spectrophotometrically).

Question 23

What are the causes of Intravascular Haemolysis?

Answer 23

1. Mismatched blood transfusion (usually ABO)

2.G6PD deficiency with oxidant stress.

3.Red cell fragmentation syndromes.

4. Some severe autoimmune haemolytic anaemias
5. Some drug- and infection-induced haemolytic anaemias.
6. Paroxysmal nocturnal haemoglobinuria
7. March haemoglobinuria
8. Unstable haemoglobin

Question 24

Which stain is used to identify Haemosiderin in a urin spun deposit?

Answer 24

Perl’s stain - Prussian blue - positive