Haematology

Question 1

Macrocytic anaemia

Answer 1

Goats milk - folate def

Pernicious anaemia - B12 deficiency

Bone Marrow Failure

• Fanconi (BM failure)
• Diamond Black fan (failure to make RBCs
• Myelodysplasia

Medications

Chemo

Question 2

Delayed umbilicard drop off associated with …

Answer 2

Leucocyte adhesion deficiency

Question 3

Clinical syndromes associated with haematological conditions and require screening

Answer 3

Hemihypertropy / Beckwith-Wiedmann Syndrome = > Wilm’s tumour

Downs = > AML/ALL

Retinoblastoma

Haemophilia = > X-Linked

HbSS

Question 4

Petechiae Differentials

Answer 4

1. Thrombocytopaneia
2. VWB Disease
3. Collagen vascular
4. Accidental
5. NAI

Question 5

Purupura DDx

Answer 5

1. Infection
   
   1. SEPSIS
2. Autoimmune
   
   1. ITP
   2. HUS
3. Vasculitis
   
   1. HSP and other vasculitides
4. Malignancy
   
   1. Leukaemia
5. Misc
   
   1. NAI
   2. SVC distribution - vomiting

Question 6

Fe deficiency

Answer 6

• Toddlers - milk (also get enteritis with this)
• Teens - Menorrhagia
• Everyone else - GI bleeding

OE

Pale, tachy, murmur

Rare: glossitis/stomatitis

Why does it matter?

• Lose IQ points

Diagnosis

FBC, ferritin, Serum Fe (varies with diet), TIBC, Fe trial

Mx

4-6mg/kg elemental Fe/day

Question 7

Fever + Petechiae

Answer 7

1. Meningococcaemia
2. Pneumonia
3. Toxic Shock Syndrome
4. Necrotising Fasciitis
5. Bacterial Endocarditis
6. DIC
7. Miliary TB
8. Vasculitis - Lupus
9. ITP
10. HSP
11. Leukaemia
12. HIV

Question 8

Features of Paediatric malignancy

Answer 8

Question 9

Malignancy Epi

Answer 9

Leukaemias 30%

CNS 22%

Lymphomas 11%

Neuroblastoma 8%

Nephroblastoma 5%

Others 22%

Question 10

Neuroblastoma

Answer 10

Epidemiology
Most common extracranial solid tumour
Approx 10% of childhood cancers, usually < 5yrs

Pathology - origins adrenal medualla and sympathetic ganglia

Presentation
Commonly with a mass
-75% abdominal
-20% mediastinal
-3% cervical
- Orbit - ecchymosis, peri-orbital oedema, strabismus, Horner’s, heterochromia iridis

Mets common - bone, bone marrow, liver, lung, skin, local invasion across the midline common

Investigation
Urine and serum catecholamines, homovanillic acid, vanillyl mandelic acid, nneuron-specific enolase

Imaging - CT/MRI, bone scan,

Tumour biopsy

Management
Surgical Excision if able

Question 11

Nephroblastoma
aka Wilm’s

Answer 11

60% present before 3 years of age

Assessment
Incidental finding of a renal mass or AP
Left > Right side, 10% bilateral
Haematuria
Poor feeding
Fever
Hypertension
Hemihypertrophy in 2%
40% have metastases at time of presentation - lung, liver, bone, brain

Some association w/ syndromes
Denys-Drash - 75% risk
WAGR - 45-60% risk
Beckwith-Wiedman 7%

Prognosis
Treatment survival up to 90% 5 yr survival depending on stage of tumour