Haematology

Question 1

What are the causes of iron deficiency anaemia?

Answer 1

Dietary insufficiency, prolonged milk consumption
Iron loss - meckels diverticulum
Inadequate absorption - chrons

Question 2

Where is iron absorbed?

Answer 2

Duodenum and jejunum

Question 3

What type of anaemia is iron deficiency?

Answer 3

Microcytic

Question 4

What is the presentation of iron deficiency anaemia?

Answer 4

Fatigue, lethargy, pallor, poor feeding, anorexia

Question 5

What eating disorder can iron deficiency anaemia present with?

Answer 5

PICA

Question 6

What is the management of iron deficiency anaemia?

Answer 6

Ferrous sulphate
Increase dietary iron - green veg, cereals, meat, egg yolk, increase vitamin C also

Question 7

What is sickle cell disease?

Answer 7

AR condition resulting in abnormal beta chains causing sickle shaped RBC that have shorter life spans and get trapped in the microcirculation

Question 8

What is the diagnosis of sickle cell anaemia?

Answer 8

Newborn blood spot test
Blood film
Hb electrophoresis

Question 9

What is a vaso-occlusive crisis?

Answer 9

Sickled RBC clog capillaries causing distal ischaemia in hands and feet often triggered by cold weather, dehydration, infection

Question 10

What is a splenic sequestration crisis?

Answer 10

RBC block blood flow in the spleen causing an acutely enlarged and painful spleen and susceptibility to infection from encapsulated bacteria

Question 11

What does a splenectomy require?

Answer 11

Life-long penicillamine prophylaxis

Question 12

What is an aplastic crisis?

Answer 12

Temporary absence of creation of new RBC triggered by parvovirus B19

Question 13

What is acute chest syndrome?

Answer 13

Vessels supplying lungs become clogged with RBC causing chest pain, SOB, fever, cough

Question 14

What can stimulate HBF?

Answer 14

Hydroxycarbamide

Question 15

Name some long-term complications with sickle cell

Answer 15

Osteomyelitis from salmonella
Cardiomegaly and arrhythmias
Ischaemic colitis
Liver dysfunction trapping sickle cells
Pigment gallstones
Occlusive crisis

Question 16

What is fanconi anaemia?

Answer 16

Rare genetic DNA repair disorder that is autosomal recessive and affects all 3 haemopoietic cell precursors

Question 17

What is the presentation of fanconi anaemia?

Answer 17

Bruising, purpura, short stature, cafe au lait spots, absent thumb, horseshoe kidney, microcephaly, cryptochidism, deafness, low set ears

Question 18

What is an FBC finding of fanconi anaemia?

Answer 18

Pancytopenia

Question 19

What is raised in fanconi anaemia?

Answer 19

Alpha fetoprotein

Question 20

What is VWD?

Answer 20

Defect in quantity or quality of VWF which links platelets to exposed underlying endothelium and binds and stabilises coagulation factor 8

Question 21

What is the presentation of VWD?

Answer 21

easy bruising, excessive bleeding, large bruises

Question 22

What is the diagnosis of VWD?

Answer 22

APTT raised
PT normal
Platelet count normal
VWF low
Factor 8 activity low

Question 23

What is the management of VWD?

Answer 23

Desmopressin - increases factor 8 activity by releasing them from storage in weibel palade bodies in the endothelium

Question 24

What is ITP?

Answer 24

Spontaneous low platelet count causing a purpuric rash caused by a type II hypersensitivity reaction 24-48 hours after viral illness (strep)

Question 25

What is haemophilia?

Answer 25

Bleeding disorder that is X-linked recessive with A being more common and a deficiency of factor 8 and B of factor 9

Question 26

what is the presentation of haemophilia?

Answer 26

Easy bleeding and bruising, haemorthioses, epistaxis

Question 27

What is the diagnosis of haemophilia?

Answer 27

APTT raised, factor 8 low, PT and VWF normal

Question 28

What is thalassaemia?

Answer 28

Genetic defect in haemoglobin protein chains - both types are autosomal recessive

Question 29

What is the presentation of thalassemia?

Answer 29

Microcytic anaemia, fatigue, pallor, jaundice, splenomegaly, gallstones, frontal bossing, poor growth

Question 30

Describe what you’d see on a slide if a child had iron deficiency anaemia.

Answer 30

Microcytic Hypochromic Low/ normal reticulocytes

Question 31

What would you see on the blood results of a child with iron deficiency anaemia?

Answer 31

Low ferritin Low serum iron Increased TIBC

Question 32

What are the side effects of ferrous sulphate?

Answer 32

Constipation and black stools

Question 33

What are the Hb, Reticulocyte, WBC values in a person with Sickle Cell Disease?

Answer 33

Low Hb Increased reticulocyte count Elevated WBC

Question 34

Describe the symptoms & blood film of beta-thalassaemia minor

Answer 34

Asymptomatic Mild anaemia Low MCV Raised Hb

Question 35

What signs + symptoms would be displayed by someone with beta-thalassaemia major?

Answer 35

Splenomegaly Failure to thrive Delayed puberty Jaundice Low MCV Progressive severe anaemia

Question 36

What is the most common form of immunologic thrombocytopenia?

Answer 36

ITP

Question 37

What is meant by a ‘coagulopathy’?

Answer 37

Errors in the clotting cascade

Question 38

What is haemolytic disease of the newborn?

Answer 38

Incompatibility between the rhesus antigens on the surface of RBCs of the mother and foetus = rhesus D antigen

Question 39

What is the presentation of haemolytic disease of the newborn?

Answer 39

Jaundice, oedema, anaemia, splenomegaly

Question 40

What is the treatment for haemolytic disease of the newborn?

Answer 40

Exchange transfusion, IV IG, Phototherapy and anti-D IG for mother

Question 41

What is iron overload?

Answer 41

Result of the faulty creation of RBC, recurrent transfusions and increased absorption of iron in the gut in response to anaemia