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Year 4 PAEDS > Haematology and Oncology 2 > Flashcards

Haematology and Oncology 2 Flashcards

1
Q

What is Henoch-Schönlein purpura (HSP)?

Is it common?

A

IgA-mediated autoimmune hypersensitivity vasculitis of childhood

Unknown cause

Most common systemic vasculitis in children (but still rare - 10/20 per 100,000 per year)

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2
Q

What are the 5 main clinical features of HSP?

A

1) Skin purpura
2) Arthritis
3) Abdo pain
4) GI bleeding
5) Nephritis

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3
Q

At what age does HSP occur?

A

90% under 10 years

Peak 4-6 years

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4
Q

What is the pathophysiology of HSP?

A

IgA immune complexes deposit in small blood vessels of skin, joints, kidneys and GIT causing inflammatory reactions

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5
Q

How does HSP present?

A

Child comes in with limp!

Most often in autumn / winter, sometimes following an URTI or GI infection

1) Mildly ill + low grade fever
2) Symmetrical, erythematous macular rash on backs of legs, buttocks, ulnar side of arms
2) Within 24hrs maculses evolve into purpuric lesions which can coalesce to resemble bruises. Raised and palpable
4) Abdo pain and bloody diarrhoea may precede rash
5) +/- n&v
6) Joint pain esp knees and ankles
7) Renal involvement in 40%
- microscopic haematuria
- proteinuria
- nephritic syndrome
- (rarely oliguria and HTN)
8) Scrotal involvement
- Can mimic testicular torsion

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6
Q

Ddx of purpuric rash

A

Thrombocytopenia

Meningococcal meningitis

Trauma

Coughing / sneezing gives pinpoint petechia by ears / above nipples

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7
Q

Ddx of HSP

A

Intussusception

Connective tissue disease eg SLE

Other causes of purpuric rashes

Other causes of glomerulopnetphritis

Other causes of GI symptoms eg IBD

Acute haemorrhage oedema of infancy

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8
Q

What is acute haemorrhage oedema of infancy?

A

Self-limiting condition presenting with fever, oedema and targeted-shaped purpura affecting face, ears and extremities

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9
Q

How is HSP diagnosed?

A

Clinically

Tests include:

1) Urinalysis
- Haeamtuira and/or proteinuria (present in 20-40%)

2) FBC
- Raised WCC with eosinophilia
- Normal or inc platelets
- Helps exclude other causes eg thrombocytopenia

3) Raised ESR
4) Raised creatinine if renal involvement
5) Raised serum IgA

6) Testicular USS
- assess possible torsion

7) Renal biopsy
- Persistent nephrotic sundrome

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10
Q

What should be performed to confirm intussusception?

A

Barium enema

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11
Q

What is the management of HSP?

A

Usually self-limiting (resolves within 4 weeks)

Supportive treatment

NSAIDs for joints
- Caution if renal insufficiency or GI symptoms

Monitor BP and urinalysis for those with proteinuria

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12
Q

What are some complicaitons HSP?

A

Renal involvement usually mild (less than 1% progress to CKD)

Rarely MI, intussusception, GI bleeding, testicular haemorrhage

Recurrence in 1/3rd within 4-6 months of initial presentation

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13
Q

What are the 4 main types of leukaemia?

A

1) Acute lymphoblastic leukaemia (ALL)
2) Chronic lymphoblastic leukaemia (CLL)
3) Acute myeloid leukaemia (AML)
4) Chronic myeloid leukaemia (CML)

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14
Q

What is the most common leukaemia?

A

ALL

Chronic rare in childhood

Leukaemia is the most common malignancy of childhood (30%)

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15
Q

What is the pathophysiology of leukaemia?

A

Malignant proliferation of white cell precursors (B or T cells) within bone marrow

These ‘blast’ cells escape into circulation and are deposited in lymphoid or other tissue

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16
Q

What is the peak age of presentation of ALL?

What age of presentation has a worse prognosis of ALL?

A

Between 2-5yrs

<2 yr or >10yr

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17
Q

How may ALL present? (8)

A

Insidious

1) Malaise
2) Anorexia
3) Pallor
4) Bruising
5) Bleeding

6) Lymphadenopathy
7) Splenomegaly
8) Bone pain

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18
Q

What may FBC show in ALL?

A

1) Anaemia
2) Thrombocytopenia
3) Raised WCC

Extremely high WCC = worse prognosis

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19
Q

What may be seen on a peripheral blood film in ALL?

A

Blast cells

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20
Q

How is a diagnosis of ALL confirmed?

A

Bone marrow aspirate
- Shows marrow infiltrated with blast cells

Cells examined by immunophenotyping and cytogenetic analysis

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21
Q

What % of ALL have specific genetic abnormalities in the leukaemic cell line? Example?

A

> 90%

eg TEL-AMLI fusion gene = 20% children with ALL

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22
Q

How can ALL be subdivide?

A 
Common = 75%
T-cell = 15%
Null = 10%
B-cell = 1%
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23
Q

What does treatment of ALL involve?

A

Chemo to induce remission - remove blast cells from circulation and restore normal marrow function

Then maintain remission

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24
Q

How is remission remained in ALL?

A

Intensification chemo

MTX or cranial irradiation protects CNS from involvement

Monthly cycles of maintenance chemo

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25
Q

What % of children achieve are successfully induced into remission in ALL?

What is the prognosis?

A

95%

Good prognosis - 80% 5yr survival

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26
Q

What are children who relapse in ALL offered?

A

High-dose chemo and bone marrow transplant

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27
Q

What may a CXR show in ALL?

A

Mediastinal mass

Lytic bone lesions

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28
Q

What are the cells in the myeloid cell line?

A

RBC
Platelets
Granulocytes
Monocytes

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29
Q

What are the cells in the lymphoid cell line?

A

Lymphocytes

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30
Q

What are WBC?

A

Lymphocyte (lymphoid)

Monocytes and granulocytes (myeloid)

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31
Q

What is lymphoma?

A

Malignancy of the lymphatic system

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32
Q

How common is lymphoma in children? It follows which other cancers?

A

3rd most common cancer

after leukaemia and brain tumours

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33
Q

What are the two main types of lymphoma?

A

Hodgkin and non-Hodgkin lymphoma (NHL)

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34
Q

How is Hodgkins lymphoma characterised?

A

Presence of multinucleate giant cell = Reed-Sternberg cells

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35
Q

What are the 4 types of NHL found in children?

A

1) Burkitt and Burkitt-like lymphoma
2) Diffuse large B-cell lymphoma
3) Anaplastic large cell lymphoma
4) Lymphoblastic lymphoma (of B or T cell origin)

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36
Q

What are some risk factors for NHL?

A

Male
Immunocompromised
DNA repair defects

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37
Q

How may NHL present?

A

Painless lymphadenopathy - neck, supraclavicular, axillary, groin

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38
Q

What investigations are done for NHL?

A 
Bone marrow aspirate
LP
Pleural and peritoneal fluid asirate
Exclusional biopsy
CT and PET scans
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39
Q

What is the treatment of NHL?

A

Same as ALL

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40
Q

What is the prognosis of NHL?

A

> 70% survival in >90% localised disease

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41
Q

What are some risk factors for Hodgkin’s lymphoma?

A 
Inc age (rare before 5yr)
More common in pt with previous EBV
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42
Q

How may Hodgkin’s lymphoma present?

A

Progressive, painless LN enlargement
- Mostly cervical and mediastinal

B symptoms = common in advanced stages

  • Fever
  • Night sweats
  • Weight loss
43
Q

How is Hodgkin’s lymphoma staged?

A

Ann Arbour staging

44
Q

What investigations are done for Hodgkin’s lymphoma?

A

CT - neck, chest, abdo, pelvis

FDT-PET scan

Bone marrow aspiration and trephine (remove 1/2cm core of bone marrow)

EBV serology

ESR

Isotope if bone involvement / b symptoms

45
Q

What is the treatment of Hodgkin’s lymphoma?

What may be required in a relapse?

What is 5yr survival?

A

Chemo
Radiotherapy

Autologous stem cell transplant

90% 5yr survival

46
Q

What is the commonest haemoglobinopathy?

A

Sickle cell anaemia

47
Q

What population does sickle cell anaemia most commonly affect?

A

Black ethnicity

48
Q

What is the cause of sickle cell anaemia?

A

Genetic defect where there is a substitution of one of the amino-acids in the global chain

Causes an unstable haemoglobin = HbS with shorter lifespan

Autosomal recessive

49
Q

What is the pathophysiology of sickle cell anaemia?

A

When HbS is deoxygenated, it forms highly structured polymers which cause brittle, spiny red cells

These occlude blood vessels = ischaemic changes

Chronic haemolytic anaemia

50
Q

How may children with sickle cell anaemia present?

A

Recurrent, acute, painful crises

Swelling of hands and feet

51
Q

What may precipitate a crisis in sickle cell anaemia?

A

Dehydration
Hypoxia
Acidosis

52
Q

Why may children with sickle cell anaemia be more susceptible to infections?

A

Repeated splenic infarction (esp in early years) can leave the child asplenic

53
Q

What may lead to dehydration in children with sickle cell anaemia?

A

Renal damage leading to a reduced ability to concentrate urine

54
Q

What is sickle cell trait?

A

Heterozygous HbS (as opposed to homozygous in sickle cell disease)

55
Q

How may sickle cell trait present?

A

Asymptomatic other than in low oxygen situations eg high altitude or under GA

56
Q

What may a physical examination of a child with sickle cell anaemia show?

A 
Chronic anaemia
Flow murmur
Jaundice
Chronic leg ulcers
Dactylitis
Splenomegaly (young child only)
Haematuria
57
Q

What are the four types of acute crisis in sickle cell anaemia?

A

1) Thrombotic
2) Sequestration
3) Aplastic
4) Haemolytic

58
Q

What is a thrombotic sickle cell acute crisis?

A

Aka painful crisis / vaso-occlusive

Various organs including bones, eg:

  • Avascular necrosis of the hip
  • Hand-foot syndrome
  • Lungs
  • Spleen
  • Brain
59
Q

What is a sequestration sickle cell acute crisis?

A

Sickling within organs eg spleen/lungs causes pooling blood with worsening anaemia

Spleen becomes enlarged causing abdominal pain

More common in early childhood

60
Q

What may result from a sequestration sickle cell crisis?

A

Severe anaemia
Marked pallor
CV collapse due to loss of effective circulating volume

61
Q

What is an aplastic acute crisis of sickle cell caused by?

A

Infection with parvovirus B19 (86%)

Other causes include viral illness

Sudden fall in Hb

62
Q

What is a haemolytic sickle cell acute crisis?

A

Rare

Fall in Hb due to an increased rate of haemolysis

63
Q

Ddx of sickle cell anaemia

A 
Leukaemia
Arthritis
Osteomyelitis
Septic artritis
Trauma
64
Q

What investigations can be done for sickle cell anaemia?

A

Peripheral blood smear:

  • Target cells
  • Poikilocytes (abnormally shaped RBC)
  • Irreversibly sickled cells

Electrophoresis:

  • HbS
  • Absent HbA

Abnormal LFTs

65
Q

What is the management of sickle cell anaemia?

A

Treatment of crises is symptomatic

Severe cases (very high HbS) - exchange transfusion

Immunisations kept up date

Daily lifelong prophylactic PO penicillin V to reduce the risk of pneumococcal disease

Antenatal screening of affected individuals

Daily oral folic acid

Bone marrow transplantation can be curative

66
Q

What are some complications of sickle cell anaemia?

A

Pneumococcal infection due to asplenism

Osteomyelitis

Renal damage

Gall stones

HF from chronic anaemia

67
Q

What is the prognosis of aplastic anaemia?

A

High mortality from sepsis <3yr

68
Q

What is priapism?

A

Persistent and painful erection of penis

Usually nocturnal with risk of long term impotence

Can occur in sickle cell crisis

69
Q

What is a neuroblastoma?

A

Commonest extra cranial tumour in children

Malignancy that can affect any part of the sympathetic nervous system most commonly the adrenal medulla

70
Q

How may a neuroblastoma present?

A

Depends on site of disease

Abdo mass +/- associated pain
Failure to thrive
Bleeding
Unwell child
Horner’s syndrome
Spinal cord, airway, bowel or vein compression
Increase in urinary catecholamine metabolites - symptoms related to excess catecholamines eg HTN, dizziness, headaches

71
Q

What is the peak age of presentation of neuroblastoma?

What is the survival of neuroblastoma in:

1) Disseminated disease
2) Low risk cases

A

First 2 years of life

1) Disseminated disease = 20-30%
2) Low risk cases = >90%

72
Q

What is tumour lysis syndrome?

A

Breakdown of large number of malignant cells either before/during chemo can lead to very high serum urate, phosphate and potassium levels

Urate precipitate in kidneys causing renal failure

73
Q

How can tumour lysis syndrome be prevented?

A

Good hydration

Allopurinol (xanthine oxidase inhibitor) or uric acid oxidase

74
Q

What investigations can be done for neuroblastoma?

A

Urine catecholamine metabolites - homovanillic acid and vanillymandelic acid

Imaging (US / MRI) - heterogenous mass with calcification

Confirm diagnosis with tumour biopsy + bone marrow biopsy of both iliac crest to check for mets (common at presentation)

75
Q

What is the management of neuroblastoma?

A

Surgery
Chemo
Radiotherapy

76
Q

What is a Wilm’s tumour? What is the mean age of onset?

A

= nephroblastoma

Commonest renal tumour in children

Mean age of onset = 3.5yrs

77
Q

How does a Wilm’s tumour present?

A

Most commonly = abdo mass

May have:
- pain
- haematuria
+/- HTN (inc renin)

78
Q

How may a Wilm’s tumour be investigated?

A

Imaging (US then CT / MRI) shows heterogenous mass causing distortion of renal architecture

79
Q

What is the most common site of metastases of a Wilm’s tumour?

A

Lungs = 20%

80
Q

How is a Wilm’s tumour managed? What is the 5yr survival?

A

Nephrecctomy + chemo

(adjuvant and/or Neo-adjuvant)

5 yr survival = 90%

81
Q

What is the genetic inheritance and pathophysiology of thalassaemia?

A

Autosomal recessive mutation leading to absence or dysfunction of the alpha or beta globing chains on haemoglobin A (HbA)

Cause haemolytic anaemia because of decreased or absent synthesis of a globin chain

82
Q

What is alpha thalassaemia?

A

Leads to impaired impaired oxygen transport and extravascular haemolysis via splenic clearance

83
Q

What are is the asymptomatic carrier state of alpha thalassaemia?

A

Alpha-thalassaemia silent carrier (minima) = one allele affected

Alpha-thalassaemia trait (minor) = two alleles affected

84
Q

How many alleles are there for alpha and beta?

A

Alpha:
4 alleles = 2 genes on Chr 16

Beta:
2 alleles = 1 gene on Chr 11

85
Q

What is the asymptomatic carrier state of beta thalassaemia?

A

Beta-thalassemia trait (minor) = one beta allele affected

86
Q

What is the symptomatic state alpha thalassaemia?

A

3 affected alleles (HbH) or 4 affected alleles (Hb Bart)

87
Q

What does Hb Bart cause?

A

Intrauterine haemolytic anaemia and hydros fettles = usually fatal

88
Q

What is the symptomatic state of beta thalassaemia?

A

Homozygous for partly functioning alleles (thalassaemia intermedia) or non-functioning alleles (thalassaemia major)

89
Q

What regions are alpha thalassaemia more common in?

A

Southeast Asia, Africa, India

90
Q

What regions are beta thalassaemia more common in?

A

Mediterranean, Middle East, Central and South Asia, China

= In UK often Greek Cypriot or Bangladeshi origin

91
Q

When does thalassaemia present?

A

Anytime from neonate to adulthood but later onset tends to be milder

92
Q

Are presenting symptoms more severe in alpha or beta thalassamia?

A

Symptoms tend to be worse in beta-thalassaemia as the most severe alpha-thalassamia don’t survive pregnancy or early life

93
Q

How may thalassaemia present?

A

1) Haemolytic anaemia
- Fatigue
- SOB
- Pallor
- Jaundice (beta)
- Cardiac flow murmur

2) Splenomegaly (alpha) or hepatosplenomegaly with abdo distension (beta)
3) FTT / growth restriction
4) Facial dysmorphia
5) Osteopenia (beta)

94
Q

Why may there be a cardiac flow murmur in thalassamia?

A

High CO

95
Q

What facial dysmorphia is seen in thalassaemia? Is it seen more in alpha or beta?

A

Fontal bossing
Maxillary hypertrophy
Large head

Commoner and more prominent in beta

96
Q

What investigations are done for thlassaemia?

A

Preconception screening for both parents in risk populations

97
Q

What would bloods show in thalassaemia?

A

Hypochromic microcytic anaemia

FBC - Low Hb, Low MCV, Low MCH
Inc reticulocytes
Blood film
Inc iron and inc ferritin in severe disease (either due to disease or due to treatment)
LFTS - increased unconjugated bilirubin (beta), inc liver enzymes if iron overload

98
Q

How is thalassaemia diagnosed?

A

Hb electrophoresis

Gap-PCR detects common deletions to confirm

99
Q

What is the management of thalassaemia?

A

Asymptomatic require no specific treatment (just avoid iron supplementation)

Transfusion if symptomatic
- Regular transfusions

Iron chelation if overload

Splenectomy / Bone marrow transplantation in severe cases

100
Q

What causes low MCV (microcytic) anaemia and what would you check? (3)

A

Low ferritin = IDA
Abnormal electrophoresis = haemoglobinopathy eg thalassaemia
High lead levels = lead toxicity

101
Q

What causes normal MCV anaemia?

A

High reticulocyte count:

1) Normal bilirubin = recent blood loss
2) Target cells + High bilirubin = haemolysis

Low reticulocyte count = chronic illness

102
Q

What is the classic sign seen in thalassaemia in skull radiographs?

A

“hair-on-end” sign

103
Q

What causes “hair-on-end” sign in thalassaemia?

A

Bone marrow hyperplasia due to insufficient erythropoiesis

104
Q

What is seen on blood smear in thalassaemia?

A

Target cells
Tear drop cells
Anisopoikilycytosis (RBC vary in size + shape)

Year 4 PAEDS (23 decks)

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