haematology

Question 1

Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease signs and symproms

Answer 1

aneamia
hepatosplenomegaly
jaundice
CCF

Question 2

Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease management

Answer 2

anti D immunoglobulin to Rh -ve mothers

intrauterine transfusion of affected fetuses

Question 3

Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease DD

Answer 3

thalassaemia

infection - toxoplasmosis, CMV

Question 4

Erythroblastosis fetalis / haemolytic disease of the newborn / rhesus disease diagnosis

Answer 4

coombs test - Rh -ve mothers tested for D antibodies

kleihauer test

Question 5

Anaemia of Prematurity cause

Answer 5

Low birth weight infants

poor erythropoietin response

Question 6

Anaemia of Prematurity signs and symptoms

Answer 6

apnoea
poor weight gain
pallor
decreased activity
tachycardia

Question 7

iron deficiency anaemia causes

Answer 7

Poor intake + increased requirement.
blood loss
malabsorption

Question 8

iron deficiency anaemia presentation

Answer 8

pallor
irritability
anorexia when Hb<50,
tachycardia
splenomegaly
if recurrent, suspect bleeding

Question 9

iron deficiency anaemia management

Answer 9

oral iron - ferrous fumarate for 3-6 months

constipation common

Question 10

Sickle Cell Disease management

Answer 10

Prophylactic penicillin 
Pneumococcal, influenza, meningococcal
vaccines
high risk of sepsis
pain management - paracetamol, NSAIDS, O2

Question 11

Sickle Cell Disease complications

Answer 11

acute anaemia (aplastic crisis)
vaso-occlusive crisis - cold, exercise, infection
cardiomegaly (high output)
strokes
sepsis
asplenia - from filtering abnormal RBC
splenomegaly

Question 12

Sickle Cell Disease aplastic crisis cause

Answer 12

parovirus B19

Question 13

Sickle Cell Disease stroke management

Answer 13

long term transfusion therapy

chelation for iron overload if transfusion long term

Question 14

β Thalassaemia Minor signs

Answer 14

asymptomatic
mild anaemia
low MCV
Raised Hb A2

Question 15

β Thalassaemia Major signs and symptoms

Answer 15

Progressive Severe Anaemia
low MCV, 
Hb F and A2 increased
Jaundice
Splenomegaly
Failure to thrive

Question 16

β Thalassaemia management

Answer 16

Genetic Counselling

Regular blood transfusion

Question 17

G6PD presentation

Answer 17

Neonatal jaundice
Chronic non-spherocytic haemolytic anaemia
Intermittent episodes of intravascular haemolysis
also dark urine (urobillinogen and haemoglobulinuria)

Question 18

G6PD what is it

Answer 18

RBC lack G6PD so exposed to oxidant induced haemolysis

Question 19

hereditary spherocytosis

Answer 19

RBC lose part of membrane, reducing surface:volume. cells become spheroidal leads to destruction

Question 20

hereditary spherocytosis presentation

Answer 20

autosomal dominant
jaundice
anaemia
splenomegaly

Question 21

Diamond Blackfan anaemia presentation

Answer 21

physical anomalies- at least 50%
• Cranio-facial
• thumb 10-20%
• Deafness
• Musculoskeletal

Question 22

Immune / Idiopathic Thrombocytopaenic Purpura (ITP)

Answer 22

platelet destruction by Ig G autoantibodes
usually post viral
acute brusing, looks dramatic rarely dangerous

Question 23

von willebrand disease causes

Answer 23

deficiency in vwf, a carrier protein for factor 8

Question 24

von willebrand disease management

Answer 24

Tranexamic acid

Question 25

haemophilia A

Answer 25

Deficiency of Factor VIII

Question 26

haemophilia B

Answer 26

Deficiency of Factor IX, ↑APTT

Question 27

haemophilia A/B presentation

Answer 27

``` X linked recessive- boys Prolonged bleeding Muscle bleeds Joint bleeds > arthritis and deformity Treatment Factor VIII/IX ```

Question 28

acute lymphoblastic leukaemia presentation

Answer 28

``` peak age 4-7 normochromic normocytic anaemia (pallor) thrombocytopenia (bleeding) leukopenia (infections) fatigue weight loss painless lumps on neck (lymphadenopathy) ```

Question 29

acute lymphoblastic leukaemia diagnosis

Answer 29

bone marrow biopsy tdt and cd10 surface markers in immunophenotyping CT/MRI and CXR

Question 30

acute lymphoblastic leukaemia complications

Answer 30

neutropenic sepsis - tazocin and gentamicin poor growth second malignancies

Question 31

acute lymphoblastic leukaemia management

Answer 31

``` Induction - vincristine, dexamethasone Consolidation and CNS treatment (if known CNS disease) Intensification Maintenance (radio / chemotherapy) ```

Question 32

fanconi anaemia

Answer 32

autosomal recessive most common cause of inherited aplastic anaemia high risk of leukaemia bone marrow transplant needed

Question 33

anaemia range

Answer 33

<110g/L

Question 34

microcytic hypochromic normocytic normochromic macrocytic ranges and causes

Answer 34

MCV <70 iron def, thalassaemia MCV 81-97 haemolysis (sickle cell) MCV >100 B12 def (pernicious/malabsorption), folate def (malnutrition)

Question 35

effects of ALL treatment

Answer 35

``` fertility growth psychological puberty intellectual ```

Question 36

ALL good prognostic factors

Answer 36

Age 2-10 Female WCC<50 No CNS disease prognosis 80%