Haemolytic Anaemias (haematology)

Question 1

What is haemolytic anaemia?

Answer 1

Anaemia that results from abnormal red cell destruction.

Question 2

What are the clinical features of specifically haemolytic anemia? (5)

Answer 2

-Jaundice
-Splenomegaly
-Bone expansion in children.
-Ankle ulcers
-Pigment gallstones

Question 3

What are the lab features of haemolytic anaemia? (4)

Answer 3

-High bilirubin
Low haptoglobin
Increased reticulocyte count
Red cell shape changes

Question 4

What are the causes of intravascular haemolysis? (5)

Answer 4

• Mismatched of blood transfusions.
  -G6PD deficiency with oxidant stress.
  -Unstable haemoglobin.
  -Some autoimmune haemolytic anaemias.
  -Drug and infection induced haemolytic anaemias.

Question 5

What defect does deficient/abnormal RBC membrane proteins cause and what effects does it cause?

Answer 5

Hereditary spherocytosis, causes potassium loss and dehydration.

Question 6

Name three clinical features of hereditary spherocytosis?

Answer 6

-Lower leg ulcers
-Increased haemolysis during infection
-Gall stones

Question 7

What are the laboratory features of hereditary spherocytosis? (4)

Answer 7

-MCHC increased
-Microsoherocytes
-Polychromasia
-Negative direct antiglobulin on coombs test.

Question 8

What are the treatment methods for hereditary spherocytosis? (4)

Answer 8

Folic acid
Blood transfusion
Splenectomy
Cholecystectomy

Question 9

What is G6DP deficiency?

Answer 9

Deficiency in the glucose 6 phosphate dehydrogenase enzyme, making the red cells susceptible to oxidant stress.

Question 10

What are the clinical and lab features of G6PD deficiency?

Answer 10

• Neonatal jaundice
  -Bite/blister cells
  -Polychromasia with acute intravascular haemolysis.

Question 11

How do we manage the G6PD deficiency? (4)

Answer 11

Consider splenectomy
Red cell transfusion
Treat infection
Stop offending drug

Question 12

What are the clinical syndromes produced by haemoglobin abnormalities? NB FOR TEST 3!

Answer 12

-Haemolysis- crystalline haemoglobins, unstable haemoglobin.
-Thalassaemia- alpha and beta resulting from reduced globin chain synthesis.
-Methaemoglobunaemia- failure of reduction.
-Familial Polycythaemia- Altered oxygen affinity.

Question 13

What are the effects ( in alpha thalassaemia) when there is:
1. Deletion of one alpha gene
2. Deletion of two
3. Deletion of three
4. All four if them deleted

Answer 13

1. Silent carrier
2. Low MCV and MCH
3. Splenomegaly, anaemia
4. Hydrops fetalis

Question 14

Three stages of beta thalassaemia and their effects (3)

Answer 14

•Minor- asymptomatic carrier
•Intermedia- moderate to severe anaemia, Splenomegaly that does not require regular transfusions.
•Major- Severe anaemia, transfusion dependent.

Question 15

How do we classify thalassaemia according to clinical management? (2)

Answer 15

Transfusion dependent thalassaemia (TDT)
Non-transfusion dependent thalassaemia (NTDT).

Question 16

What are the clinical features of thalassaemia?

Answer 16

-Bone expansion, intercurrent infection, failure to thrive.

Question 17

What are the laboratory features of thalassaemia? (4)

Answer 17

-Hypochromic microcytic anaemia
- Raised red cell count
-Hypercellular bone marrow
-DNA testing shows a specific mutation

Question 18

How do we manage thalassaemia? (4)

Answer 18

-Iron chelation therapy
-Immunise- Hepatitis B
-Splenectomy
-Bone marrow transplant

Question 19

What characterises the warm type of autoimmune haemolytic anaemia?

Answer 19

Red cells coated with IgG alone or complement, the coated membrane is lost making the cell more spherical.

Question 20

What are the causes of the warm type autoimmune haemolytic anaemia? (4)

Answer 20

-Idiopathic (50%)
-Viral infections
-Malignancies
-Drugs

Question 21

Clinical and lab features of the warm type of autoimmune haemolytic anaemia (5)

Answer 21

-Pallor or jaundice
-Splenomegaly
-Microspherocytes, polychromasia, +/- normoblasts.
-IgG or IgG and complement
-Direct positive antiglobulin test (coombs)

Question 22

Treatment methods for warm type autoimmune haemolytic anaemia (5)

Answer 22

-Cortocosteroids
-High dose IV immunoglobulins
-Splenectomy
-Blood transfusion
-Folic acid

Question 23

Treatment of cold type autoimmune haemolytic anaemia (4)

Answer 23

-Avoid exposure to cold
-Folic acid supplements
-Plasma exchange
-Blood transfusion