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Paediatrics > Paediatrics: Haematology + Oncology > Flashcards

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
Q

What are the common features of HL and who does it present in?

A

Presents in childhood

Painless lymphadenopathy of cervical and mediastinal LN
B symptoms - night sweats, fever, weight loss

26
Q

What is the staging system for HL?

A

Ann-Arbor

27
Q

What is the staging system for NHL?

A

St.Jude

28
Q

What is the staging system for AML?

A

FAB (french american british)

29
Q

How do you differentiate definitely between HL and NHL?

A

HL = Reed-Stenberg cells (multinucleated)

30
Q

What is the treatment for lymphoma

A

Chemotherapy

Radiotherapy

31
Q

when does NHL typically present?

A

Adolescents for paediatrics

However median age is actually 50

32
Q

What is the most common childhood cancer?

A

Acute lymphoblastic leukaemia (ALL)

33
Q

What are the symptoms of ALL?

A
  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
Q

What is the treatment for ALL?

A

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
Q

What are the symptoms of AML?

A

General: Weight loss and fatigue

1. Lymphadenopathy (

36
Q

What are the blood results of ALL?

A
  1. FBC - Low Hb, Neut, platelets

2. Leukaemic blast cells

37
Q

What are the different classfications of brain tumours in paediatrics?

A
  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
Q

What are the signs of raised ICP?

A

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
Q

What is the gold standard for diagnosis a brain tumour?

THINK LOGICALLY

A

MRI head obviously

40
Q

What are the different types and locations of brain tumour? (5)

A
  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
Q

Where does Craniopharyngioma develop from? what are the symptoms (think triad)

A

Remanant of Rathke’s pouch

  • Raised ICP
  • Visual field loss
  • Pituitary dysfunction
42
Q

In which chromosome is a and b Globulin genes codeD?

A

two a globulin genes on each chromosome 16

two b globulin genes on a single chromosome 11

43
Q

What are the feature of a-thalassaemia based on severity

A
  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
Q

What are the features of b-thalassaemia trait and major?

A

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
Q

When does B-thalassamia major typically occur and why?

A

12-18 months when HbF drops, but there is no is made HbA –> anaemia

46
Q

What are the symptoms of Wilms tumour?

A

Abdominal mass (U/L)
Flank pain
Haematuria
Fever + Anorexia

47
Q

What are the features of neuroblastoma and where does it commonly present?

A

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
Q

What markers would be raised in neuroblastoma?

A

Urinary HVA and VMA (catecholamines)

49
Q

What is the Hb type common at birth?

A

HbF (a2 g2)

50
Q

What is the Hb type common after 1yo?

A

HbA (a2 b2)
2.5% HbA2 (a2 d2)
1% HbF (a2 g2)

Paediatrics (10 decks)

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