Haematology

Question 1

Anaemia pathology

Answer 1

Decreased RBC production (due to ineffective erythropoiesis eg iron deficiency OR red cell aplasia Increased RBC destruction (haemolysis) Blood loss

Question 2

Main causes of iron deficiency anaemia

Answer 2

Inadequate intake Malabsorption Blood loss

Question 3

Iron intake requirements of 1 year old vs men + women

Answer 3

1 year old = 8mg a day Men = 9mg a day Women 15mg a day

Question 4

Foods for infants with iron

Answer 4

Breast milk Infant formula Cows milk Cereals introduced at weaning

Question 5

What increases + inhibits iron absorption?

Answer 5

Increases = vit C Inhibits = tannin in tea, high fibre foods

Question 6

S+S anaemia

Answer 6

Asymptomatic until Hb drop below 6 Fatigue SOB FTT Pale Pica

Question 7

Microcytic changes in anaemia

Answer 7

Microcytic cells Hypochromic cells Low MCV + MCH

Question 8

S+S haemophilia in infants

Answer 8

Bleeding - intracranial haemorrhage May present when starting to walk - haemoarthroses

Question 9

Investigations for haemophilia

Answer 9

FBC + blood film PT - measures factors 2, 5, 7 + 10 APTT - measures factors 2, 5, 8, 9, 10, 11 + 12

Question 10

Normal changes in neonates clotting factors

Answer 10

All clotting factors except 8 and fibrinogen are lower

Question 11

What can be given in haemophilia A to manage it without the use of blood products?

Answer 11

Desmopressin (DDAVP)

Question 12

Complications of treatment for haemophilia

Answer 12

Inhibitors (ab) develop, reducing effectiveness of treatment

Question 13

Pathology of Henoch Schonlein purpura?

Answer 13

Vasculitis Usually preceded by URTI GI petechiae = GI bleeds Renal injury due to deposition of IgA

Question 14

HSP RF

Answer 14

2-10 years Males more common Peaks in winter

Question 15

S+S HSP

Answer 15

Rash over buttocks + extensor surfaces of arms + legs Swelling of face, hands + feet Periarticular oedema Colicky abdo pain Haematemesis + malaena Glomerulonephritis

Question 16

Management of HSP

Answer 16

Supportive Resolves in 1-3 weeks

Question 17

Pathology of sickle cell anaemia

Answer 17

HbS inherited instead of HbA Caused by mutations on B-globin gene Sickled cells have decreased lifespan, resulting in blood vessel occlusion + ischaemia Exacerbated by low O2 tension, dehydration + cold

Question 18

S+S sickle cell

Answer 18

Recurrent haemolysis Thrombosis in mesenteric, intracranial + bone = severe pain

Question 19

Sickle cell disease vs trait

Answer 19

Disease = nearly normal Hb, fewer painful crisis May develop retinopathy + osteonecrosis Trait = asymptomatic

Question 20

Investigations for sickle cell

Answer 20

Guithre test

Question 21

Management of sickle cell

Answer 21

Hydroxyurea

Daily penicillin

BM transplant

Question 22

Pathology of thalassaemia

Answer 22

Microcytic anaemia Haemaglobinopathy that causes intrinsic abnormalities of RBC Haemolysis due to absent HbA a-thalassemia = caused by deletions in a-globin gene b-thalassemia = caused by mutations in Bglobin gene

Question 23

S+S thalassaemia

Answer 23

Haemolysis leads to anaemia, hepato + splenomegaly, Increased levels of Unconjugated bilirubin Excess urinary urobilinogen

Frontal bossing

Question 24

Diagnostic clues to haemolysis

Answer 24

Increased reticulocyte count = polychromasia (lilac colour) Raised urinary urobilinogen

Question 25

What does purpura suggest?

Answer 25

Platelet <30

Question 26

What is purpura fulminans?

Answer 26

Consequence of vasculitis in skin, can lead to necrosis

Happens after meningitis + VZV due to production of antiviral ab which react + inactivate protein S

Question 27

What does a high WCC + low platelets indicate?

Answer 27

Leukamia

Question 28

What does PT + APTT measure?

Answer 28

PT = factor 2, 5, 7, 10

APTT = factor 2, 5, 8, 9, 10, 11, 12 (intrinsic + extrinsic)

Question 29

Management of haemophilia

Answer 29

Recombinant factor concentrate

Virally inactivated plasma products next

Desmopressin in Haem A