Paediatric Haematology

Question 1

IDA dietary advice

Answer 1

Dark green veg
iron fortified bread
meat
apricots
prunes
raisins

Question 2

IDA Mx

Answer 2

explore cause
Supplement:
- PO ferrous sulphate 200mg 2/3times daily, continue if corrected after 3m to build up stores (alt. PO ferrous fumarate or ferrous gluconate)
Monitor

Question 3

Advice in IDA taking Fe

Answer 3

Adverse effects: constip. diarr., impaction, GI irritation, nausea
Can be minimised by taking supplment w/food or reducing frequency

Question 4

Monitoring in IDA

Answer 4

Check Hb 2-4wk after starting Fe (should rise by 2g/100mL over 3-4wks)
If level sufficient check at 2-4m to ensure stable
check adherance (compliance issues)
Once Hb and RBC indices normal:
- Continue Fe treatment for 3m, monitor every 3m for 1yr
- recheck after 1yr
- consider continuing as prophylaxis for at risk:
- recurring, iron poor diet, malabs, menorrhagia, gastrectomy

Question 5

Hereditary spherocytosis

Answer 5

Neonates; supportive +/- blood trx, Folate supplementation, Consider phototherapy or exhange trx if jaundiced
Infants, children, adults:
- supportive +/- RBC trx
- folate 2-5mg PO OD
- PO penicillin (pneumoc.)
Aplastic crises (B19) - blood trx
- ?splenectomy (w/vaccines for NHS)
- ?cholecystectomy bc. gallstones common

Question 6

G6PD deficiency

Answer 6

Sx of acute haemolysis: jaundice, pallor, dark urine (inform parents of these)
Avoidance of drugs and foods
Acute haemolysis: supportive care + folate
Bloood trx and renal support if necessary

Question 7

Sickle Cell Disease prophylaxsis

Answer 7

Immunisation against encapsulated organisms (pneumoniae, HiB)
Daily PO penicillin
Daily PO folate
avoid cold, dehydration, excessive exercise, hypoxia

Question 8

Treating acute sickle cell crises

Answer 8

Oral and IV analgesia (avoid morphine <12y)
Good hydration
Infection w/Abx
Oxygen
Exchange trx for acute chest syndrome, priapism, and stroke

Question 9

Treatment of chronic problems in sickle cell

Answer 9

> 3 admissions in 12m for acute chest syndrome or vaso-occlusive crises could benefit from: hydroxycarbamide (stimulated HbF production)
Monitor for WBC suppression if prescrbied
BMT in severe cases

Question 10

Prognosis of SCD

Answer 10

can cause prem death due to Cx

50% of pts w/most severe disease die before 40

Question 11

Beta-thalassemia

Answer 11

beta-thalassemia major is fatal w/o reg. blood trx
aim to maintain Hb >100 to reduce growth failure/prevent bone deformation
Iron chelation
Good adherence a/w living beyond 40y
BMT is only cure (only if HLA identical sibling)
PN diagnosis: CVS offered to heterozygous parents

Question 12

Cx of beta-thal

Answer 12

cardiac failure
liver cirrhosis
diabetes
infertility 
growth failure

Question 13

Fanconi anaemia

Answer 13

BMT

Question 14

Haemophilia Mx

Answer 14

A: factor 8 concentrate
B: factor 9 concentrate
Acute bleeds are treated w/factor concentrates and antifibrinolytics by prompt IV infusion (aminocaproic acid, TXA )
analgesia and physio if bleed in joint
AVOID:
IM injections
aspirin/NSAIDs

Question 15

Cx of haemophilia

Answer 15

antibodies to recombinant factors can develop
transfusion related infections
difficult vascular access

Question 16

Prophylactic Factor 8

Answer 16

for ALL children w/severe haemophilia A to reduce chronic joint damage

Question 17

DDAVP + haemophilia

Answer 17

mild haemophilia A as it stimulates endogenous FVIII and vWF release

Question 18

Von Willebrand Dx Mx

Answer 18

Depends on type and severity
- type 1: DDAVP (caution if <1y because hyponat. can cause seizure)
more severe vWD treated w/plasma derived factor 8 concetrate
AVOID in WD:
IM injection
Aspirin
NSAIDs

Question 19

Immune thrombocytopaenic purpura Ix

Answer 19

FBC, blood smear
in 80% of children disease is acute, benign, self limiting, resolve within 6-8w
Mx at home
Treatment indicated if evidence of major bleeding (intracranial/GI) or persistent minor bleeding eg epistaxsis

Question 20

Life or organ threatening bleed in ITP

Answer 20

IVIG + corticosteroid + plt. transfusion

consider anti-fibrinolytics

Question 21

Newly dx child w/ITP

Answer 21

Asymptomatic or minor bleed:
observation (most will achieve normal plt)
Major:
corticosteroids
IVIG or Anti-D IG

Question 22

Child w/chronic ITP

Answer 22

Mycophenolate mofetil
rituximab
eltrombopag (TPO agonist)
2 line: splenectomy

Question 23

DIC in children

Answer 23

treat cause (usually sepsis)
supportive care
restore physiological coaguation pathways (heparin)
Replacement therapy - factors and plts
Antithrombin and protein C concentrates esp in severe meningococcal septicaemia
antifibrinolytics in small subset