Haem

Question 1

Managing sickle cell crisis

Answer 1

Analgesia
Rewarming
Oxygen
Antibiotics
IV fluids

Question 2

Indications for exchange transfusion in SCD

Answer 2

Chest crisis
Stroke
Priapism

Question 3

Indications for top up transfusion in SCD

Answer 3

Significant drop in Hb from baseline

Question 4

In child with limp + bone pain what is the most important diagnosis to exclude?

Answer 4

1: acute leukaemia
2: septic arthritis

Question 5

Managing ITP

Answer 5

2-10 yrs
Self limiting
No continued bleeding = outpatient
Continued bleeding + haemodynamic instability = fluid bolus + close monitoring
Platelets given will just be destroyed
Don’t give unless bleeding life threatening

Question 6

Most important management priority in newly diagnosed AML

Answer 6

Prophylactic hyper hydration, allopurinol/rasburicase+ electrolyte monitoring req from diagnosis to prevent tumour lysis syndrome:

• hyperkalaemia
• hyperphosphataemia
• gout
• fluid overload/ dehydration
• raised urea + creatinine

Question 7

Complications of childhood malignancy

Answer 7

Short stature
Infertility
Educational difficulties
Haematological malignancy

Question 8

Type 1 vWD

Answer 8

AD
Partial defiance of vWF
Mild phenotype

Question 9

Type 2 vWD

Answer 9

AD

Defective vWF

Question 10

Type 3 vWD

Answer 10

AR

Complete vWF deficiency

Question 11

Fanconi’s anaemia

Answer 11

AR

Aplastic anaemia

Question 12

Pica

Answer 12

Eating non food substances
Due to severe iron deficiency
Microcytic anaemia + low ferritin
Prolonged breastfeeding + delayed weaning
Iron stores sufficient for 4 months, then req supplementation

Question 13

Other consequences of delayed weaning

Answer 13

Hypocalcaemia

Poor weight gain

Question 14

ITP diagnosis

Answer 14

Platelets

Question 15

ALL

Answer 15

80% of childhood leukaemias
Splenomegaly, bruises, lethargy, pallor
More common in boys
Pk incidence 5 yrs
Higher incidence in caucasians

Question 16

AML

Answer 16

Far less likely that ALL however some conditions incr risk
Downs ALL= AML
Bloom’s syndrome
Neurofibromatosis

Question 17

Beta thalassemia major

Answer 17

AR - point mutation Chr11
Complete lack of beta globin
No functioning HbA, reliant on HbA2 + HbF
Decr HbF in 1st yr -> FTT due to severe microcytic anaemia, normal ferritin
Mediterranean + Middle eastern ethnicity

Question 18

Beta thalassemia minor

Answer 18

AR 
Heterozygous for beta chain mutation on Chr 11
Mild hypochromic, microcytic anaemia 
Usually asymptomatic
Incr levels HbA2 + HbF

Question 19

Clinical features of beta thal major

Answer 19

FTT, lethargy, pallor + jaundice
Hepatosplenomegaly
Skull bossing, long bone deformity (excessive intramedullary haematopoiesis)

Question 20

Treating beta thal major

Answer 20

Regular blood transfusions aiming to maintain Hb > 100
Death inevitable in first few years of life without treatment
Or allogenic BM transplant
Iron chelation therapy (desferrioxamine) required to prevent iron overload

Question 21

Alpha thalassemia

Answer 21

South East Asia
One gene corruption: asymptomatic
Two gene corruptions: mild hypochromic anaemia
Three corruptions: HbH disease(beta chain tetramers)
Four corruptions: HbBarts (gamma chain tetramers) death in utero

Question 22

Causes of microcytic anaemia

MCV

Answer 22

Thalassemias
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia

Question 23

Causes of normocytic anaemia

MCV 85-105

Answer 23

Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia

Question 24

Macrocytic anaemia

MCV>105

Answer 24

Failure of DNA synthesis
Megaloblastic anaemia: vit B12/folic acid defic/pernicious anaemia
Liver disease
Hypothyroidism

Question 25

How would you treat ALL?

Answer 25

Supportive: transfusion + abx
Chemotherapy:
Remission induction
Consolidation
Maintenance
Can take a number of years
High risk of neuro disease = CNS prophylaxis: intrathecal methotrexate + radiotherapy
Good prognosis with appropriate therapy

Question 26

How is malaria diagnosed?

Answer 26

Thick blood film: parasite detection
Thin blood film: detect species and determination of parasitaemia (% erythrocytes infected)
Signet ring inclusions

Question 27

Complications of malaria

Answer 27

Haemoglobinuria: due to severe haemolysis - black water fever
Acute renal failure
Cerebral malaria: fits + coma, main cause of death

Question 28

Malaria treatment:

Answer 28

Oral quinine
IV quinine
SE: tinnitus, deafness + nausea
OD: heart block
Exchange transfusion with parasitaemia >10%

Question 29

G6PD triggers

Answer 29

Fave (broad) beans
Anti malarials + sulphonamide abx
Infections
Supportive treatment, occasionally transfusions required

Question 30

Hodgkin’s lymphoma

Answer 30

Asymmetrical painless lymphadenopathy
Painful on alcohol ingestion
B sx
Reed Sternberg cells
Early: CT scan + radiotherapy
Advanced: CT scan, radiotherapy + chemo

Question 31

Prognosis of Hodgkin’s lymphoma

Answer 31

70% chance of cure

Incr age / systemic sx= poorer prognosis

Question 32

What skeletal infections are children with ScD at greater risk of?

Answer 32

Septic arthritis
Osteomyelitis
Strep or salmonella

Question 33

Causes of pica (eating non-nutritive substances)

Answer 33

Iron deficiency

Lead poisoning

Question 34

Features of lead poisoning

Answer 34

Encephalopathic signs
Pica, blue line on gums
Hypochromic microcytic anaemia
Basophilic stippling
Measure blood lead levels
Treatment= chelation with EDTA

Question 35

Pernicious anaemia

Answer 35

AI attack of gastric parietal cells = no intrinsic factor ->
Vit B 12 deficiency, normal ferritin
Macrocytic anaemia
Anti parietal antibodies (Fewer antiIF antibody)
Peripheral neuropathy, smooth tongue, angular stomatitis, depression, dementia, subacute degen of the spinal cord

Question 36

Dx and treatment of pernicious anaemia

Answer 36

Dx: schilling test
Rx: 3 monthly IM hydroxycobalamin a vit b12 analogue