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Paediatrics: Haematology + Oncology Flashcards

1
Q

What are the symptoms of HSP?

A

Triad of:

  1. Abdominal pain
  2. Arthralgia/Arthritis - of large joints knees, ankles
  3. Palpable, papular non-blanching rash - symmetrical around buttocks and legs (trunk sparing)

Also:

  • Oedema of dorm hand, scrotum and periodical
  • Haematemesis
  • Melaena
  • UTI - haematuria, proteinuria (commonly caused by nephrotic syndrome or glomerulonephritis)
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2
Q

What is HSP?

When and who does it present?

A

Systemic vasculitis mediated by IgA/IgG complexes

Presents commonly in pre-pubescent boys - 3-10 years old

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3
Q

What is the treatment for HSP?

A
  1. Self-limiting - symptoms typically resolve in 6 weeks
  2. NSAIDs and Steroids - treats abdominal and arthritic pain
  3. High dose corticosteroids + cyclophosphamide if severe
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4
Q

What are the possible complications of HSP?

A
  1. Perforation of bowel following appendicitis or intussusception
  2. Nephrotic syndrome or glomerulonephritis
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5
Q

What are common symptoms of Fe deficiency anaemia?

A

Often asymptomatic - infants can tolerate surprisingly low levels of Hb before becoming clinically symptomatic

Pallor - conjunctiva
Dyspnoea 
Fatigue/Lethargy
Poor feeding 
Neuro - poor cognitive motor and sensory function, mood or behaviour change, irritable, pica
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6
Q

What are the key investigative findings of Fe deficiency anaemia? (2)

A
  1. FBC - Low Hb (<6-7g/l), MCV, MCH, MCHC, platelets, serum ferritin, Increased total iron binding capacity (confirms def)
  2. Film - microcytic, hypo chromic RBCs
    (can be macro and microcytic if missed FE/B12 Folate deficiency)
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7
Q

What is the primary cause of Fe deficiency anaemia?

A

Inadequate dietary intake

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8
Q

What are the secondary causes of Fe deficiency anaemia?

A 
Malabsorption - Coeliac, IBD 
Thalassaemia A (middle east, asian, mediterranean), Thal B (African)
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9
Q

What is the treatment for Fe deficiency anaemia?

Tip: be logical, don’t try and medicalise everything

A

Education

  1. Breast feed - low Fe content but high absorption
  2. Bottle feed - add Fe supplements
  3. Encourage high Fe foods - red meat, eggs, vitamin C rich foods (green beans, broccoli)
  4. Cows milk - high protein but poor absorption
  5. Avoid - milk and tea (tannins inhibit Fe uptake)
  6. Cereals

Pharmacology

  1. Folic acid (ferrous salt)
    - 3 months
    - if Hb does not increase, consider thalassaemia
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10
Q

What is the cause of haemophilia?
Who does it affect?
When does it present?

A

X-linked recessive disorder
Males only
Neonates and infancy

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11
Q

What are the two types of haemophilia? Which is more common?

A 
Haemophilia A - factor VIII deficiency (common) 
Haemophilia B (Christmas disease) - factor IX deficiency (rare)
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12
Q

What are the symptoms of haemophilia?

Tip: categorise into mild, moderate, severe

A

Mild (40%)
- bleed following surgery

Moderate (20%)

  • bleed following minor trauma
  • easily bruised
  • bleeding gums and nose

Severe (<1%)

  • recurrent bleed into joints and muscles (knees, ankles, hips, elbows)
  • -> arthritis and arthralgia (painful, hot, swollen, tender, reduced RoM, unable to weight bear)
  • -> degenerative bones (joint bleed)
  • -> compartment syndrome (muscle bleed)
  • -> Intracranial bleed following head trauma
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13
Q

What are the investigative findings for haemophilia?

A
  1. Haematology
    - Increased APTT
    - Reduced factor VIII or IX
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14
Q

What is the treatment for haemophilia?

A
  1. IV recombinant VIII or IX
    - Prophylaxis (alt days) and treatment of haemorrhage
  2. Tranexamic acid - if mouth bleed
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15
Q

What must you absolutely avoid in haemophilic patients?

A
  1. NSAIDs - decrease function of platelets
  2. IM injections - including Vit.K at birth
  3. Contact sport
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16
Q

When does sickle cell anaemia (HbSS) present and in whom?

A

6 months - 3 years

Afro-carribeans and middle-east

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17
Q

What factors trigger sickle cell exacerbations or crisis? (3)

A

Cold temperature
Dehydration
Oxygen desaturation

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18
Q

What are the symptoms of sickle cell anaemia?

A

Dactylitis - swollen and painful hands and feet
Infection - encapsulated organisms
Jaundice and icterus
Anaemic symptoms - SoB, fatigue, pallor, dizzy
Splenic sequestration - due to RBCs packed in spleen unable to escape

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19
Q

How is sickle cell anaemia diagnosed?

A
  1. Blood film:
    - Sickle cells
  2. FBC:
    - Reticulocytosis
    - Hb < 5-9 g/l
  3. Hb electrophoresis (diagnostic)
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20
Q

What are the management options for acute sickle cell crisis?

A
  1. ABCDE - keep warm
  2. Oxygen - aim > 95%
  3. Fluids - 150% of normal maintenance fluids
  4. Abx - Cephalosporin (if febrile and +ve cultures)
  5. Blood transfusion
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21
Q

What is a potential curative treatment for sickle cell anaemia?

A

Bone marrow transplant

22
Q

What is the maintenance treatment of sickle cell anaemia?

A
  1. Penicillin V - prevent infection from encapsulated organisms
  2. Hydroxycarbamide
  3. Folic acid
  4. Immunisations
23
Q

When does aplastic anaemia occur in sickle cell disease?

A

Typically post parvovirus infection

24
Q

What are the features of sickle cell crisis?

A
  1. Vaso-occlusive disease - painful joints and muscles
  2. Avascular necrosis - hips and humerus
  3. Pneumococcal sepsis - common in 1st three years
  4. Stroke - common at 5-10 yo
  5. Acute chest syndrome - dyspnoea, chest pain, cough, fatigue
  6. Priapism
25
What are the common features of HL and who does it present in?
Presents in childhood Painless lymphadenopathy of cervical and mediastinal LN B symptoms - night sweats, fever, weight loss
26
What is the staging system for HL?
Ann-Arbor
27
What is the staging system for NHL?
St.Jude
28
What is the staging system for AML?
FAB (french american british)
29
How do you differentiate definitely between HL and NHL?
HL = Reed-Stenberg cells (multinucleated)
30
What is the treatment for lymphoma
Chemotherapy | Radiotherapy
31
when does NHL typically present?
Adolescents for paediatrics However median age is actually 50
32
What is the most common childhood cancer?
Acute lymphoblastic leukaemia (ALL)
33
What are the symptoms of ALL?
1. General - Weight loss and Fatigue 2. Bone marrow infiltration - Anaemia - fatigue, SoB, pallor - Neutropoenia - infection - Thrombocytopoenia - bleed and bruising 3. Reticulocyte infiltration - Hepatosplenomegaly - lymphadenopathy 4. Others - Pleural effusion - Scrotal swelling - Airway obstruction - Painful joints and bones
34
What is the treatment for ALL?
SVADM induction (4 weeks) 1. Steroids - hydrocortisone or prednisolone 2. Vincristine - weekly 3. L-Asparginase 4. Daurubicin 5. IT methotrexate - day 18 8 weeks of Chemotherapy
35
What are the symptoms of AML?
General: Weight loss and fatigue | 1. Lymphadenopathy (
36
What are the blood results of ALL?
1. FBC - Low Hb, Neut, platelets | 2. Leukaemic blast cells
37
What are the different classfications of brain tumours in paediatrics?
1. Infratentorial (60%) - presents with raised ICP, headache, nausea + vomiting, cerebellar ataxia 2. Supra-tentorial - presents with raised ICP, focal CNS neuro deficit, hypothalamic/pituitary dysfunction, visual imp. 3. Primary spinal - Cord compression symptoms 4. CNS mets -
38
What are the signs of raised ICP?
U/L unresponsive pupils Headache - worse on coughing, straining, leaning forward, lying down Nausea and vomiting Irritable Confused and altered level of consciousness Papillioedema
39
What is the gold standard for diagnosis a brain tumour? THINK LOGICALLY
MRI head obviously
40
What are the different types and locations of brain tumour? (5)
1. Astrocytoma (40%) - presents anywhere, includes optic glioma - juvenile - slow growing, good prog post-op - non-juvenile - poor prog 2. Medulloblastoma - midline of posterior fossa - Ataxia, headache, vomiting, risk of spinal mets 3. Ependmymoma - posterior fossa and may also involve ventricles 4. Brainstem glioma - poor prognosis, early childhood - Ataxia and CN signs 5. Craniopharyngioma - develops from remnant of Rathke's pouch - Raised ICP, pituitary dysfunction, visual field loss
41
Where does Craniopharyngioma develop from? what are the symptoms (think triad)
Remanant of Rathke's pouch - Raised ICP - Visual field loss - Pituitary dysfunction
42
In which chromosome is a and b Globulin genes codeD?
two a globulin genes on each chromosome 16 | two b globulin genes on a single chromosome 11
43
What are the feature of a-thalassaemia based on severity
1. One a-chain absent - Asymptomatic 2. Two a-chain absent - Microcytic, hypochromic, Hb often normal but may mimic Fe deficiency anaemia (Low MCV, MCH, Hb) 3. Three a-chain absent (HbH disease) - Reticulocytosis, microcytic, hypo chromic - Jaundice and splenomegaly 4. 4 a-chain absent - Hb Bart's (G4) causes death in utero due to hydrops Bart's foetalis
44
What are the features of b-thalassaemia trait and major?
Trait - Asymptomatic - Microcytic, hypochromic - Low MVC, MCH, Hb Major - Frontal skull bossing - Hepato-spleno-mgealy - Severe anaemia - Developmental failure - Reticulocytosis, target cells, nucleated RBC - HbF but not HbA
45
When does B-thalassamia major typically occur and why?
12-18 months when HbF drops, but there is no is made HbA --> anaemia
46
What are the symptoms of Wilms tumour?
Abdominal mass (U/L) Flank pain Haematuria Fever + Anorexia
47
What are the features of neuroblastoma and where does it commonly present?
Typically presents in neural crest of adrenal gland (common) or sympathetic chain ``` Abdominal mass - painless Abdominal distension - due to hepatomegaly Abdominal discomfort Proctosis Horner's syndrome Joint pain, limp, paraplegia Watery diarrhoea Sweating ±Fever ```
48
What markers would be raised in neuroblastoma?
Urinary HVA and VMA (catecholamines)
49
What is the Hb type common at birth?
HbF (a2 g2)
50
What is the Hb type common after 1yo?
HbA (a2 b2) 2.5% HbA2 (a2 d2) 1% HbF (a2 g2)

Paediatrics (10 decks)

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