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Oncology & Haematology Flashcards

1
Q

Main site of haemopoiesis in fetus and postnatal?

A

Fetus - Liver

Postnatal - BM

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2
Q

Hb and WBC, and platelet counts as neonate?

A 
High Hb (14-21g/dL) - drops to 10 by 2m
High WBC (10-20x10^9/L)
Platelet similar to adult
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3
Q

Hb in anaemia in

a) neonate
b) 1-12m
c) 1-12y

A

a) <14g/dL
b) <10g/dL
c) <11g/dL

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4
Q

3 mechanisms anaemia may arise?

A

Reduced RBC production
Increased RBC destruction
Blood loss

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5
Q

Causes of anaemia in children?

A

Reduced RBC production

  • Red cell aplasia
  • Ineffective erythropoiesis (Fe/folate/B12 deficiency)

Increased RBC destruction

  • RBC membrane disorders (hereditary spherocytosis)
  • RBC enzyme disorders
  • Haemoglobinopathies (thalassaemias, sickle cell)
  • Immune (haem disease of newborn)

Blood loss

  • Fetomaternal bleeding
  • Chronic GI blood loss (Meckel’s)
  • Inherited bleeding disorders (vWillebrands)
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6
Q

MCV in

a) iron deficiency anaemia?
b) folic acid deficiency?

A

a) low

b) high

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7
Q

Causes of iron deficiency anaemia?

A

Inadequate intake
Malabsorption
Blood loss

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8
Q

At what level of Hb does anaemia become symptomatic?

A

6-7g/dL

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9
Q

Main causes of microcytic anaemia?

A
  1. Iron deficiency
  2. beta-thalassaemia trait
  3. alpha-thalassaemia trait
  4. Anaemia of chronic disease (e.g. renal failure)
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10
Q

Management of iron deficiency anaemia?

A
  • Dietary advice and supplementation with oral iron
  • Iron supplementation until 3m after Hb normal
  • Hb should rise 1g/dL per wk with good compliance
  • If no response → look for malabsoprtion (eg Coeliac) or chronic blood loss (eg Meckel diverticulum)
  • Blood transfusion should never be necessary for dietary iron deficiency (even if Hb =2)
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11
Q

Lifespan of RBC?

A

120d

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12
Q

Main causes of haemolysis in children?

A
  • -> Intrinsic abnormalities of RBCs
    1. RBC membrane disorders eg hereditary spherocytosis
    2. RBC enzyme disorders eg G6PD deficiency
    3. Haemoglobinopathies eg thalassaemia
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13
Q

Clinical features of haemolysis?

A 
Anaemia
Hepatomegaly
Splenomegaly
Increased unconjugated bilirubin
XS urinary urobilinogen
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14
Q

Diagnosis of haemolysis on blood film?

A
  • Raised reticulocyte count

- Abnormal appearance of RBCs

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15
Q

Genetics of hereditary spherocytosis?

A
  • Usually autosomal dominant

- No FH in 25% - new mutation

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16
Q

Pathophysiology of hereditary spherocytosis?

A
  • Mutations in genes for proteins of RBC membrane (mainly spectrin, ankyrin or band 3)
  • → RBC loses part of membrane when passes through spleen
  • This causes cells to become spheroidal
  • → Destruction in microvasculature of spleen
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17
Q

Clinical features of hereditary spherocytosis?

A
  • Often suspected because FH
  • Clinical manifestations highly variable - may be asymptomatic
  • Jaundice – usually develops during childhood, may be intermittent; may cause severe haemolytic 
jaundice in 1st few days of life

  • Anaemia –mild (Hb 9–11 g/dl), but Hb may fall during infections
  • Mild/mod splenomegaly – depends on rate of haemolysis
  • Aplastic crisis – uncommon, transient (2–4w), caused by parvovirus B19 infection
  • Gallstones – due to increased bilirubin excretion
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18
Q

How is hereditary spherocytosis diagnosed?

A

Blood film usually diagnostic

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19
Q

Management of hereditary spherocytosis?

A
  • Most have mild chronic haemolytic anaemia and only treatment required = oral folic acid (raised folic acid requirement secondary to increased RBC production)
  • Splenectomy beneficial but only indicated for poor growth or troublesome sx of anaemia (e.g. severe tiredness, loss of vigour)
  • Usually deferred until after 7y because of risks of post­ splenectomy sepsis
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20
Q

Inheritance of G6PD deficiency?

A

X-linked

Usually affects males

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21
Q

Clinical features of G6PD deficiency?

A
  • Neonatal jaundice – onset in 1st 3d of life
  • Acute haemolysis – precipitated by:
    – Infection = most common precipitating factor
    – Certain drugs (antimalarials, antibiotics, analgesias)
    – Fava beans (broad beans)
    – Naphthalene in mothballs
  • Haemolysis predominantly intravascular
  • → Associated with fever, malaise and passage of dark urine - contains Hb as well as urobilinogen
  • Hb level falls rapidly and may drop <5 g/dl over 24–48 h 

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22
Q

How is G6PD deficiency diagnosed?

A
  • B/w episodes, almost all pts have completely normal blood picture and no jaundice or anaemia
  • Diagnosis made by measuring G6PD activity in RBCs
  • During haemolytic crisis, G6PD levels may be misleadingly elevated due to higher enzyme conc in reticulocytes → produced in increased numbers in response to destruction of RBCs
  • Repeat assay required in steady state to confirm diagnosis
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23
Q

Management of G6PD deficiency?

A
  • Parents given advice about signs of acute haemolysis (jaundice, pallor and dark urine) and provided with a list of drugs, chemicals and food to avoid
  • Transfusions rarely required, even for acute episodes
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24
Q

What is

a) Sickle cell anaemia?
b) HbSC disease?
c) Sickle beta-thalassaemia?
d) Sickle cell trait?

A

a) Pts homozygous for HbS (virtually no HbA, small amount HbF) - most severe
b) HbS from 1 parent, HbC from other (no HbA)
c) HbS 1 parent, B-thalassaemia other –> no HbA - same picture as SC anaemia
d) HbS 1 parent, HbA other - carrier

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25
Q

What is sickle cell anaemia exacerbated by? (3)

A
  • Low O2 tension
  • Cold
  • Dehydration
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26
Q

Manifestations of sickle cell anaemia? (6)

A 
Anaemia
Infection - increased susceptibility
Painful crises - vaso-occlusive
Acute anaemia - haemolytic/aplastic/sequestration crisis
Priapism
Splenomegaly
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27
Q

Long-term problems of sickle cell anaemia?

A
  • Short stature and delayed puberty
  • Stroke and cognitive problems
  • Adenotonsillar hypertrophy
  • Cardiac enlargement – from chronic anaemia
  • Heart failure – from uncorrected anaemia
  • Renal dysfunction
  • Pigmented gallstones
  • Leg ulcers – uncommon in children
  • Psychosocial problems – education and behavioural difficulties
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28
Q

Which infections are people with sickle cell more susceptible to?

A

Pneumococci
H Influenzae
–> Due to hyposplenism due to microinfarction in spleen

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29
Q

Prophylaxis in sickle cell anaemia?

A
  • Full immunisation
  • Daily oral penicillin
  • OD folic acid
  • Avoid cold, dehydration, XS exercise, stress, hypoxia
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30
Q

Management of acute crises in SC anaemia?

A
  • Painful crises → oral/IV analgesia according to need (pos opiates) and good hydration
  • Infection → abx
  • O2 if sats reduced
  • Exchange transfusion indicated for acute chest syndrome, stroke and priapism
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31
Q

Management of chronic problems in SC anaemia?

A
  • If recurrent hosp admissions for vaso­occlusive crises or acute chest syndrome may benefit from hydroxyurea
  • → Drug increases HbF prodn and helps protect against further crises
  • Requires monitoring for SEs, esp WBC suppression
  • Most severely affected children (1–5%) who have a stroke or don’t respond to hydroxyurea may be offered BM transplant
  • → Only cure but can only be safely done if child has HLA­identical sibling who can donate BM – cure rate 90% but 5% risk of fatal transplant­related complications
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32
Q

2 types of beta-thalassamia?

A

Major
- No HbA due to 2 abnormal beta-globin genes

Intermedia

  • Small amount HbA or large amount HbF produced
  • Only 1 abnormal beta-globin gene
33
Q

Clinical features of beta-thalassamia?

A
  • Severe anaemia - transfusion dependent, from 3–6m of age and jaundice
  • FTT/growth failure
  • Pallor
  • Jaundice
  • Splenomegaly
  • Hepatomegaly
  • Bossing of skull
  • Maxillary overgrowth
34
Q

Management of beta-thalassamia?

A
  • Lifelong monthly blood transfusions (fatal w/o)
  • Aim for Hb >10g/dL
  • Iron chelation from 2-3yo
  • BM transplant if HLA identical sibling
35
Q

Problems with repeated blood transfusion due to iron overload? (5)

A
  • Cardiac failure
  • Liver cirrhosis
  • Diabetes
  • Infertility
  • Growth failure
36
Q

Complications of long-term blood transfusion in children? (4)

A
  • Iron overload
  • Antibody formation (10%)
  • Infection (<10%)
  • Venous access (common)
37
Q

3 types of alpha-thalassamia?

A
  • Major
  • HbH disease
  • Alpha-thalassaemia trait
38
Q

What is alpha thalassaemia major?

A
  • Most severe α­-thalassaemia, (also known as Hb Barts hydrops fetalis)
  • Deletion of all 4 α­globin genes → no HbA (α2β2)
  • Occurs mainly in SE Asian origin
  • Presents in mid­trimester with fetal hydrops (oedema and ascites) from fetal anaemia
  • → Always fatal in utero or within hours of delivery
  • Only long­term survivors are those who have received monthly intrauterine transfusions until delivery followed by lifelong monthly transfusions after birth
  • Diagnosis is made by Hb electrophoresis or Hb HPLC (high­performance liquid chromatography), which shows only Hb Barts
39
Q

What is HbH disease?

A
  • Only three α­globin genes deleted
  • Affected children have mild–moderate anaemia
  • Occasional pts are transfusion­ dependent
40
Q

What is alpha-thalassaemia trait?

A
  • Deletion of 1 or 2 α­globin genes
  • Usually asymptomatic and anaemia mild or absent
  • RBCs may be hypochromic and microcytic → may cause confusion with iron deficiency
41
Q

5 main components of normal haemostasis?

A
  1. Coagulation factors
  2. Coagulation inhibitors
  3. Fibrinolysis
  4. Platelets
  5. Blood vessels
42
Q

What is mucous membrane and skin haemorrhage characteristic of?

A

Platelet disorder or von Willebrand disease

43
Q

What is bleeding into joints/muscles characteristic of?

A

Haemophilia

44
Q

What are haemophilia A and B?

A 
A = coagulation factor VIII deficiency
B= coagulation factor IX deficiency
45
Q

Clinical features of haemophilia?

A
  • Most present end of 1st y of life
  • 40% present as neonates –> ICH, bleeding post-circumcision or prolonged bleeding from venepuncture/heel-prick
  • Bleeding episodes most frequent into joints/muscles
46
Q

How is haemophilia severity classified?

A

Mild –> FVIII:C 5-40%, bleed after surgery

Mod –> FVIII:C 1-5%, bleed after minor trauma

Severe –> FVIII:C <1%, bleed spontaneously into joint/muscles

47
Q

Management of haemophilia?

A
  • Recombinant FVIII or IX
  • Given IV whenever bleeding
  • Raising level to 30%sufficient to treat minor bleeds
  • 100% for surgery and maintained at 30-50% for 2w after
  • Injections every 12h or by infusion
  • Pts/children taught to administer at home
  • Prophylactic FVIII given to all with severe disease to raise level >2% → better joint function in later life
  • Desmopressin may allow for mild haemophilia A to be managed without blood products (stimulates FVIII + vWF release) – ineffective in type B
  • Specialised physio
  • IM injections, aspirin and NSAIDs avoided in all pts
48
Q

What are the 2 roles of von willebrand factor?

A
  1. Facilitates platelet adhesion to damaged endothelium

2. Acts as carrier protein for FVIII:C, protecting it from inactivation and clearance

49
Q

Clinical features of von willebrand disease?

A
  • Bruising
  • XS, prolonged bleeding after surgery
  • Mucosal bleeding such as epistaxis and menorrhagia
  • In contrast to haemophilia, spontaneous soft tissue bleeding such as large haematomas and haemarthroses uncommon
50
Q

Management of von willebrand disease?

A

DDAVP

More severe –> plasma-derived FVIII

IM injections, aspirin and NSAIDs avoided

51
Q

Name 3 acquired bleeding disorders?

A
  • Vit K deficiency (mainly neonates)
  • Liver disease
  • Thrombocytopenia (DIC, ITP etc)
52
Q

Define thrombocytopenia

A

Platelet count <150 × 10^9/L

  • Severe <20 (spontaneous)
  • Mod 20-50 (mild trauma/ ops)
  • Mild 50-150 (major op/severe trauma)
53
Q

Commonest cause of thrombocytopenia in childhood?

A

ITP

- Usually caused by destruction of circulating platelets by anti-platelet IgG autoantibodies

54
Q

Clinical features of ITP?

Prognosis?

A
  • Most b/w 2 -10y
  • Onset often 1–2w after viral infection
  • In majority, short hx of days-wks
  • → Petechiae, purpura +/or superficial bruising
  • Epistaxis/other mucosal bleeding
  • Profuse bleeding uncommon, despite platelet count often <10 × 10-9/L
  • Intracranial bleeding = serious but rare complication (0.1–0.5%) → mainly if long period of severe thrombocytopenia

In 80% → disease is acute, benign and self­limiting, usually remitting spontaneously within 6–8w

55
Q

If suspect ITP, what would lead to a BM examination? (5)

A
  • Anaemia
  • Neutropenia
  • Hepato­splenomegaly
  • Lymphadenopathy
  • Treated with steroids (may mask ALL)
56
Q

ITP management?

A
  • Most at home with no treatment (even platelet<10)
  • Treatment if major bleeding (e.g. intracranial or GI)
  • Treatment if affecting daily life (epistaxis/menorrhagia)
  • Oral prednisolone
  • IV anti­D
  • IV immunoglobulin
  • → All have significant SEs
  • Platelet transfusions for life­threatening haemorrhage as they raise platelet count only for few hrs
57
Q

How many have chronic ITP and what is the management?

A
  • 20% persists beyond 6m
  • In most just supportive treatment
  • Drug if chronic persistent bleeding affecting daily life
  • Eg rituximab
  • Splenectomy if drugs fail
58
Q

What is DIC?

A

Disorder characterised by coagulation pathway activation → diffuse fibrin deposition in micro­vasculature and consumption of coagulation factors and platelets

59
Q

Commonest causes of activation of coagulation in DIC? (4)

A
  • Sepsis
  • Meningococcal septicaemia
  • Shock due to circulatory collapse
  • Extensive tissue damage from tissue damage/burns
60
Q

Clinical features of DIC? (3)

A
  • Bruising
  • Purpura
  • Haemorrhage
  • -> Whilst critically ill
61
Q

What blood test abnormalities are there in DIC?

A
  • Thrombocytopenia
  • Prolonged PT
  • Prolonged APTT
  • Low fibrinogen
  • Raised fibrinogen degradation products & D-dimers
  • Microangiopathic haemolytic anaemia
62
Q

Causes of purpura?

A

Non-thrombocytopaenic

  • HSP
  • Sepsis
  • Trauma

Thrombocytopaenic

  • ITP
  • Leukaemia
  • DIC
63
Q

Illnesses that may indicate splenectomy? (4)

A
  • Hereditary spherocytosis
  • Lymphoma
  • Chronic ITP
  • Trauma to spleen with uncontrolled bleeding
64
Q

Vaccinations needed to be given prior to splenectomy? (4)

A
  • Pneumococcus
  • Hib
  • Meningococcus
  • Influenza
65
Q

Which leukaemia most common in children?

A

ALL (80%)

66
Q

Clinical presentation of ALL?

A
  • Peaks at 2-5y
  • Mostly insidious presentation (several wks)

Gen –> malaise anorexia

BM infiltration

  • anaemia (pallor, lethargy)
  • neutropenia (infection)
  • thrombocytopenia (bruising, petechiae, epistaxis)
  • bone pain

Reticulo-endothelial infiltration

  • hepatosplenomegaly
  • lymphadenopathy

Other organ infiltration

  • CNS–> headaches, vomiting, nerve palsies
  • Testes –> testicular enlargement
67
Q

Ix for ALL?

A
  • FBC
  • BM examination
  • CXR (look for mediastinal mass characteristic of T cell disease)
68
Q

2 types of ALL?

A 
Common subtype (75%)
T-cell subtype (15%)
69
Q

5 stages of treatment regimes for ALL?

A 
Induction of remission
Consolidation and CNS protection
Interim maintenance
Delayed intensification
Continuing maintenance
70
Q

Short term SEs of chemotherapy?

A
  • Hair loss
  • Anaemia
  • Infection
  • Bruising
  • Sore mouth
  • N+V
  • Mood changes
  • Wt gain
71
Q

Long term SEs of chemo?

A
  • Delayed puberty
  • Reduced fertility
  • Reduced growth
  • Neurotoxicity, hepatotoxicity, renal toxicity, cardiotoxicity, pulmonary toxicity
  • Secondary cancer
  • Psychological effects
72
Q

Which lymphoma more common?

A

Non-Hodgkin lymphoma (80%)

Yet Hodgkin more common in adolescence

73
Q

Common clinical features of neuroblastoma?

A

Mostly <5yo

  • Pallor
  • Wt loss
  • Abdo mass (most have at presentation)
  • Hepatomegaly
  • Bone pain
  • Limp
74
Q

What is neuroblastoma?

A

Tumour of neural crest tissue in adrenal medulla and sympathetic NS

75
Q

Ix for neuroblastoma?

A
  • Urinary catecholamines
  • MRI
  • Confirmatory biopsy
  • BM sample/ bone scan for metastatic disease
76
Q

What is Wilm’s tumour?

A

Nephroblastoma

  • Commonest renal tumour of childhood
  • Mostly <5yo
  • V rare >10yo
77
Q

Clinical features of Wim’s tumour?

A

Common
- Abdo mass

Uncommon

  • Abdo pain
  • Anorexia
  • Anaemia (haemorrhage into mass)
  • Haematuria
  • HTN
78
Q

Associations with Wilm’s tumour?

A

Overgrowth syndromes

Trisomy 18

79
Q

Most common presenting feature of retinoblastoma? (2)

A

White pupillary reflex replaces normal red one

Squint

Paeds (15 decks)

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