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Paediatrics > Haematology and Oncology > Flashcards

Haematology and Oncology Flashcards

1
Q

How does iron deficiency cause anaemia? -it leads to ineffective ___

A

-ineffective erythropoiesis/red cell production

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2
Q

Name 3 causes of anaemia due to red cell aplasia in children;

A
  • parovirus b19 infection
  • diamond blackfan anaemia (congenital red cell aplasia)
  • Fanconi anaemia
  • aplastic anaemia
  • leukaemia
  • transient erythroblastopenia of childhood
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3
Q

What are the main causes of iron deficiency anaemia (IDA) in children?

A
  • inadequate intake
  • malabsorption
  • blood loss
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4
Q

Name a couple of sources of iron intake in an infant:

A
  • breastmilk (low content but 50% absorbed) /infant supplemented formula
  • cow’s milk (higher content but only 10% absorbed)
  • solids introduced at weaning e.g. (fortified) cereals (only 1% absorbed)
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5
Q

What are the clinical fts of IDA in children?

A
  • asymptomatic until Hb <70g/L
  • tiring easily
  • infants feed more slowly
  • pallor: conjunctivae, tongue, palmar creases
  • ‘pica’ e.g. soil, chalk, gravel eating
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6
Q

What blood results are diagnostic of iron deficiency anaemia?

A
  • microcytic hypochromic anaemia (low MCV and MCH)

- low serum ferritin

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7
Q

What are 2 other differentials (other than IDA) in a child with a microcytic anaemia:

A
  • beta thallassaemia trait (NB: alpha thallass have microcytic MCV but most are not anaemic
  • anaemia of chronic disease
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8
Q

Management of IDA in children:

A
  • dietary advice

- oral iron supplementation (sytron or niferex) these should rise the Hb by 10g/L per week

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9
Q

If after oral iron, anaemia is not improving and history suggests there is a non-dietary cause of IDA, what differentials could be investigated?

A
  • malabsorption due to coeliac disease

- chronic blood loss due to Meckel’s diverticulum

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10
Q

Sub-clinical iron deficiency (e.g. low ferritin but not anaemic) is controversial to treat suggest an adv, a disadv and a solution in the management of this:

A
  • adv: if untreated, IDA can cause behavioural/intellectual function decline
  • disadv: if orally treated, there is a risk of poisoning which is v toxic
  • solution: give dietary advice to increase iron intake and its absorption
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11
Q

What pattern of inheritance is sickle cell disease?

A

Autosomal recessive

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12
Q

HbS (sickle cell) arises as a result of a point mutation in codon _ of the __ ___ gene.
This causes a change in amino acid encoded from glutamine to ____

A

codon 6 of the beta-globin gene

–> valine

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13
Q

State the main types of sickle cell disease(4):

A
  • sickle cell anaemia (HbSS)
  • HbSC disease
  • Sickle B-thalassaemia
  • Carriers/Sickle Cell Trait
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14
Q

Pathogenesis of sickle cell disease in one sentence. (HbS … –> sickle shape)

A

-HbS polymerises within RBCs forming rigid tubular spiral bodies which deform red cells into a sickle shape

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15
Q

How do the irreversibly sickled cells lead to organ/bone ischaemia? What exacerbates this?

A
  • they have a reduced lifespan
  • they may be trapped in microcirculation (–>vaso-occlusion)
  • leads to ischaemia
  • exacerbated by: low O2, dehydration, cold
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16
Q

What is one of the most important factors that affects severity of Sickle Cells disease and varies between phenotypes affecting disease severity?

A

-amount of HbF (most pts have levels <1% but some genetic variations with 15%+ have much less severity)

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17
Q

Prophylaxis is part of the management of sickle cell disease, what may this involve?

A
  • full immunisation (susceptible to infections esp. encapsulated e.g. strep pneumo, Hib due to functional asplenia)
  • pneumococcal, Hib and meningococcal vaccinations
  • daily oral penicillin in childhood (protects vs. all pneumococcal subgroups)
  • OD oral folic acid (increased demand arises from the chronic haemolytic anaemia)
  • avoid exposure to cold/dehydration/undue stress/hypoxia
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18
Q

Why do sickle cell pts have hypospenism/functional asplenism? What is an emergency related to this?

A
  • secondary to chronic sickling and microinfarction in the spleen
  • sequestration crisis: sudden splenic enlargement, abdo pain and circulatory collapse from accumulation of sickled cells in spleen
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19
Q

Priapism in sickle cell children needs to be treated quickly with _____ ____ as it may lead to _____ of the ___ ___ and subsequent ___ ___

A
  • exchange transfusion
  • fibrosis of the corpus cavernosa
  • erectile impotence
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20
Q

Name 5 long term complications of sickle cell disease:

A
  • short stature, delayed puberty
  • stroke, cognitive problems (–> poor concentration/school performance)
  • adenotonsillar hypertrophy (can –> OSA –> nocturnal hypoxaemia –>vaso-occlusive crisis/stroke)
  • cardiac enlargement (from chronic anaemia)
  • heart failure, renal dysfunction, leg ulcers, psychosocial problems, pigment gallstones (more bile produced)
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21
Q

How should acute vaso-occlusive crisis in sickle cell be managed?

A
  • oral/IV analgesia
  • good hydration (oral/IV)
  • treat infection with abx
  • give o2 if sats low
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22
Q

Give 2 indications for exchange transfusion in pts with sickle cell disease?

A
  • priapism
  • acute chest syndrome
  • stroke
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23
Q

What medication increased HbF and is used for sickle cell children with recurrent crisis/acute chest syndrome to protect vs. further crises? What is the main side effect of this drug that should be monitored for?

A
  • Hydroxycarbamide

- SE risk of white blood cell suppression

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24
Q
  • what is the last stage of treatment available in sickle cell pts with stroke/no response to hydroxycarbamide?
  • what are the big risks/difficulties with this?
A
  • bone marrow transplant (90%cure rate)
  • need an HLA-identical sibling to donate their bone marrow
  • 5% risk of fatal transplant associated complications
25
Q

What is the prognosis for children with sickle cell in terms of dying from a bacterial infection?
Long term life expectancy approximately..?

A
  • 3% mortality in childhood

- 50% severe sickle cell pts die by 40yrs

26
Q

What test is used to screen for sickle cell neonatally and prenatally?

A
  • Guthrie test using the dried blood spots in the 1st week of life
  • Pre-natally chorionic villus sampling
27
Q

Sickle cell pts with HbSC have ~normal Hb level and have fewer painful crises (vs HbSS), what should be checked periodically in adolescence, due to a risk of ___ ___? they are also prone to develop ______ of the hips and shoulders.

A
  • check eyes periodically, risk of proliferative retinopathy

- osteonecrosis

28
Q

Sickle cell trait are carriers but is asymptomatic, why are they screened prior to a general anaesthetic?

A

-to make sure any additional effort to prevent hypoxia is made as sickling is theoretically poss if carriers are exposed to low o2 tension

28
Q

Sickle cell trait are carriers but is asymptomatic, why are they screened prior to a general anaesthetic?

A

-to make sure any additional effort to prevent hypoxia is made as sickling is theoretically poss if carriers are exposed to low o2 tension

29
Q

Hemopoiesis main site is the ____ whereas after birth the production is from the ___ ____

A
  • liver

- bone marrow

30
Q

What reason is behind why neonates are asymptomatic with sickle cell and signs only develop later in 1st year of life?

A

-higher HbF in first few months is protective

31
Q

all Hb has an alpha-2 chain. What is the second chain in the following:

  • HbF
  • HbA
  • HbA2
A
  • HbF: gamma-2
  • HbA: beta-2
  • HbA2: delta-2
32
Q

Neuroblastoma dx can be aided by the presence of what tumour marker in the urine?

A

Catecholamine excretion (VMA and HVA acids)

33
Q

What tumour marker can be used to monitor treatment response in the context of germ cell and liver tumours

A

-alpha fetoprotein

34
Q

Imatinib is a targeted therapy, it is a tyrosine kinase inhibitor that targets what? In context of what malignancies?

A
  • targets the BCR-ABL fusion that causes the Philadelphia chromosome +:
  • ALL
  • and CML
35
Q

Monoclonal abs can be used in cancer rx e.g. rituximab (anti-CD20) is used for l____ and
-anti-GD2 is used for treatment of high-risk n_____

A
  • rituximab for lymphoma

- anti-GD2 for neuroblastoma

36
Q

Fever + neutropenia in a immunosuppressed child should -> immediate admission and bs-abx. Cancer associated opportunistic infections include:

  • what pneumonia in children w leukaemia?
  • disseminated fungal infection caused by?
  • and infection of central venous catheters by what type of organism?
A
  • Pneumocystis Jiroveci
  • Aspergillosis or candidiasis
  • coagulase-negative staphylococcal infection
37
Q

ALL is 80% of leukaemia in children, what is the next most common?

A

Acute non-lymphocytic leukaemia (ANLL) and AML

38
Q

ALL peak age is 2-5yrs, presentation is variable. FBC is often abnormal how? And what ix is need to confirm dx and identify prognostic info?

A
  • FBC: anaemia, thrombocytopenia, circulating leukemic blast cells
  • BM exam
39
Q

10% of children at the time of ALL diagnosis have what abnormality? Which is why all patients should have what screen at presentation?

A
  • DIC

- clotting screen

40
Q

Why is a LP and a CXR performed in the ix of ALL?

A
  • LP: to identify disease in the CSF

- CXR: to identify a mediastinal mass characteristic of T-cell disease

41
Q

Before starting ALL rx, anaemia may need correction, platelets given and infection treated. What is given when starting rx to protect renal function vs the effects of rapid cell lysis?

A
  • hydration

- allopurinol or urate oxidase

42
Q

Based on the general effects, BM, RES, CNS and testes infiltration suggest a sx each for ALLs presentation

A
  • general: malaise, anorexia
  • BM: bone pain, bruising, infection, pallor, lethargy
  • RES: hepatosplenomegaly, lymphadenopathy
  • CNS: headache, vomiting, nerve palsy
  • Testes: testicular enlargement
43
Q

Suggest 2 poor prognostic factors for ALL:

A
  • age <1 or age >10yrs
  • v high tumour load (high WCC 50+)
  • cytogenic/molecular genetic abnormalities
  • poor initial response to chemo
44
Q

Neuroblastomas arise from neural ___ ____ in the adrenal ____ and sympathetic nervous system. It can spontaneously regress.

A

-neural crest tissue in adrenal medulla

45
Q

Neuroblastoma is most common

A
  • <5yrs

- benign = ganglioneuroma

46
Q

How do most children (<5yrs) present with a neuroblastoma?

A
  • an abdominal mass usually (but mass can lie anywhere along sympathetic chain neck-> pelvis)
  • bone pain, BM suppression, weight loss, malaise are often presenting sx due to metastatic disease
47
Q

Amplification of which oncogene predicts aggressive behaviour of neuroblastomas?

A

-MYCN

48
Q

Management of Neuroblastoma in:

  • pts w localised primaries w no mets:
  • metastatic disease in older children:
A
  • primaries: surgery alone

- mets: chemo, autologos stem cell rescue, surgery, radiotherapy

49
Q

Wilms tumour aka ____blastoma. How do most present?

A
  • nephroblastoma
  • present with a large abdo mass often found incidentally in a well child
  • presents with heamaturia
50
Q

How is Wilm’s tumour investigated, what would you see?

A
  • Ultrasound, CT/MRI

- intrinsic renal mass distorting the structure

51
Q

How is Wilms tumour treated?

A
  • initial chemo

- followed by delayed nephrectomy

52
Q

Suggest 3 diagnostic clues that would point towards red cell aplasia as the cause of an anaemia:

A
  • low reticulocyte count despite low Hb
  • normal bilirubin
  • negative Coombs test (DAT)
  • absent red cell precursors on BM exam
53
Q

Suggest 3 diagnostic clues that would point towards haemolysis as the cause of an anaemia:

A
  • raised reticulocyte count & increased RBC precursors in BM
  • unconjugated bilirubinaemia
  • increased urinary urobilinogen
  • abnormal red cells on blood film e.g. sphere, sickle, hypochromic…
  • positive DAT Coombs test (if immune cause)
54
Q

What is the inheritance of most Hereditary Spherocytosis

NB: 25% are sporadic new mutations

A

Autosomal Dominant

55
Q

Mutations coding for what possible things leads to the clinical disease of Hereditary Spherocytosis

A
  • coding for the RBC membrane proteins:
  • Spectrin
  • Ankyrin
  • or Band 3
56
Q

Mutations in the spectrin/ankyrin/band 3 RBC membranes in Hereditary Spherocytosis leads to destruction of cells where and how?

A

due to these mutations, RBC loses part of membrane as passes in spleen -> spheroidal shape

  • mutations make the RBCs becomes less deformable
  • so they are destroyed in the microvasculature of the spleen
57
Q

Suggest 2 common sx of Hereditary Spherocytosis and 2 poss complications, one due to an infection, one due to increased excretion of a certain thing

A
  • jaundice
  • anaemia (usually mild but falls low during infections)
  • splenomegaly
  • aplastic crisis (caused by parovirus b19)
  • gallstones (due to increased bilirubin excretion)
58
Q

What is the main 2 oral medications used in Hereditary Spherocytosis management?

A
  • oral folic acid

- life long daily oral penicillin prophylaxis

Paediatrics (12 decks)

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