Haematology and Oncology

Question 1

What are some clinical features of Sickle Cell Anaemia?

Answer 1

Splenic dysfunction

Frequent infections

Haemturia

Heart failure

Osteonecrosis

Anaemia

Pain

Question 2

What is the most severe form of Alpha Thalassaemia called?

Answer 2

Hydrops Fetalis

Question 3

What is Alpha Thalassaemia?

Answer 3

Disease of the alpha-globulins in haemoglobin.

Sometimes the are formed abnormally or not formed at all.

Question 4

True or False:

There are more than three types of Sickle cell Anaemia.

Answer 4

True.

There are numerous types of Sickle Cell Anaemia - these include hemoglobin SS disease, hemoglobin SC disease, hemoglobin S-β0-thalassemia, hemoglobin S-β+ thalassemia etc

Question 5

Name three Haemoglobinopathies

Answer 5

Alpha Thalassaemia

Beta Thalassaemia

Sickle Cell Anaemia

Question 6

What is the most severe form of Beta Thalassaemia?

Answer 6

BEta Thalassaemia Major, which is caused by a person having two defective alleles for the disease (because it is a autosomal recessive disorder)

Question 7

Name two medicines used in treatment of Iron Overload?

Answer 7

Deferoxamine

Deferasirox

Question 8

Give two examples of membranopathies

Answer 8

Hereditary Elliptocytosis and Hereditary Spherocytosis

Question 9

Name three bleeding disorders and what causes the disease

Answer 9

Haemophillia A - disorder of factor 8

Haemophillia B - (Christmas Disease) disorder of factor 9

Von willebrand Disease - (three types) disorder of von willebrand factor

Question 10

What are 4 clinical features of beta thalassaemia?

Answer 10

Anaemia - tiredness and lethargy

Iron Overload (haemochromatosis)

Osteopenia (bony fractures and weakness)

Hepatosplenomegaly

Question 11

What is the primary cause of a Sickle Cell crisis?

Answer 11

The sickle shaped RBCs blocking off microvasculature in the body.

Question 12

Name one medicine which is used in the management of Sickle Cell Anaemia

Answer 12

Hydroxycarbamide (hydroxyurea)

Question 13

What three groups are haematological disorders split into?

Answer 13

Haemoglobinopathies

Membranopathies

Enzyme problems

Question 14

What are the three important parts of treatment of beta thalassaemia major?

Answer 14

Regular blood transfusions

Splenectomy - reduces the regularity of the transfusions but increases the risk of infections

Chelation - giving supplments to prevent iron overload

Question 15

What are membranopathies?

Answer 15

Disorders which affects the cell membrane of the red blood cell, thus causing problems with the function of the cell.

Question 16

What are the three types of Von Willebrand Disease?

Answer 16

Type 1 - Partial VWF made

Type 2 - Abnormal VWF made

Type 3 - No VWF made

Question 17

What is Beta Thalassaemia?

Answer 17

It is a disorder of the beta-globulins of the haemoglobin protein.

Question 18

What are the 6 clinical signs of problems in paediatric haematology?

Answer 18

Jaundice

Failure to thrive

Petechiae/Purpura

Bleeding

Anaemia

Panctyopenia

Question 19

Name one haematological disorder caused by problems withe the enzymes

Answer 19

G-6-PD enzyme deficiency

It is a x-linked disorder - the ezyme helps to protect the RBCs from damage and premature destruction.