Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

4th Year > Paeds - Haem/Onc > Flashcards

Paeds - Haem/Onc Flashcards

1
Q

What is the most common renal tumour affecting children?

A

Wilms tumour = a Nephroblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are some clinical features of a nephroblastoma?

A
  • abdo mass found incidentally on eg. bathing or dressing a well child
  • may present with abdo swelling, abdo pain, fever, haematuria, HTN
  • Examination = abdo distension + palpable renal mass
  • Other associated features = periorbital ecchymosis (dark circles under eyes), abdo mass that crosses the midline, signs of bone marrow infiltration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How would you investigate a nephroblastoma?

A
  • FBC, U&E, etc
  • Urine dip for the haematuria
  • US for the renal/abdo mass
  • CT/MRI for staging
  • Biopsy for definitive diagnosis
  • Risk scoring if relavent
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How would you manage a nephroblastoma?

A
  • supportive care initially (hydration, nutrition, treat any co-existing infection)
  • Stage 1 + 2 tumours = Surgery (Nephrectomy)
  • Higher stages = Chemo first to reduce vol of malignant tissue before Surgery (Nephrectomy)
    • follow up patients who have had chemo as late SE is cardiotoxicity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is leukaemia?

A

Cancer of a particular line of the stem cells in the bone marrow.

  • genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell
  • Excessive production of a single type of cell can lead to suppression of other cell lines (underproduction)
  • This results in a pancytopenia (Triad of low RBC, WBC and Platelets)
    • ie Anaemia + Leukopenia + Thrombocytopaenia

Cell line can be myeloid or lymphoid.

Classified by if they are chronic (slow progression) or acute (fast progression)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the most common types of leukaemia and what are the peak ages?

A

Most common is Acute Lymphoblastic Leukaemia (ALL)

  • Peak age 2-3 years old

Acute Myeloid Leukaemia (AML) is next most common

  • Peak age is under 2 years old

Chronic Myeloid Leukaemia (CML) is rare

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are some risk factors for leukaemia?

A
  • Radiation exposure (eg. abdo xray during pregnancy)
  • Down’s syndrome
  • Kleinfelter Syndrome
  • Noonan syndrome
  • Fanconi’s anaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How would leukaemia present?

A
  • Unexplained fever
  • failure to thrive
  • weight loss
  • night sweats
  • pallor (anaemia)
  • petechiae + abnormal bruising (thrombocytopaenia)
  • generalised lymphadenopathy
  • unexplained or persistent bone/ joint pain
  • hepatosplenomegaly
  • persistent fatigue + vague abdo pain
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How would you diagnose suspected leukaemia?

A
  • NICE =
    • refer any child with unexplained petechiae or hepatomegaly
    • If leukaemia is suspected based on the clinical presentation, do an urgent FBC within 48 hours
  • FBC (shows anaemia, leukopenia, thrombocytopenia, high numbers of abnormal WBC)
  • Blood film (shows blast cells)
  • Bone marrow biopsy
  • Lymph node biopsy
  • CXR for staging
  • CT scan for staging
  • LP
  • Genetic analysis and immunophenotyping of abnormal cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How would you manage leukaemia?

A
  • Referred to paediatric oncology MDT
  • Chemotherapy
    • SE = stunted growth and development, immunodeficiency and infections, neurotoxicity, infertility, cardiotoxicity, failure to treat
  • Radiotherapy
  • Bone marrow transplant
  • Surgery
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is Lymphoma?

A

Malignancy of the lymphatic system.

Commonly divided into Hodgkins lymphoma and non-Hodgkins lymphoma.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What clinical features would you see in suspected lymphoma?

A

Hx

  • hx of Epstein-Barr Virus
  • hx of Immunosuppression (eg. hx of solid organ transplant or chemo)
  • a visible or palpable mass
  • “B symptoms” = weight loss, night sweats, fevers
  • Vague symptoms of malignancy like lethargy, anorexia

Exam

  • Non-tender lymphadenopathy
    • thought mediastinal or intra-abdo lymph nodes are not always visible or palpable
    • tender lymph nodes + hx of recent infection = suspect reactive lymphadenopathy or lymphadenitis if an abscess has formed (fluctuant tender lymph nodes)
  • Mediastinal lymphadenopathy = cough, wheeze, difficulty breathing, occasionally Superior Vena Cava Obstruction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How would you investigate suspected lymphoma?

A
  • FBC to exclude infection
  • U&Es (tumour lysis syndrome can occur when chemo is commenced = phosphorus, K+ and Ca2+ released into blood that can cause kidney damage)
  • Lactate Dehydrogenase (LDH) is usually elevated
  • USS of the area to identify other nodes + help with biopsy
  • CXR if symptoms of mediastinal node involvement
  • full body CT for staging
  • Lymph Node Biopsy for definitive diagnosis
  • Staging
    • higher stages = more groups of lymph nodes or organs involved
    • add a B (eg. stage 3b) if “B symptoms” are present
      • associated with a worse prognosis at all stages
        *
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How would you manage lymphoma?

A

a mediastinal mass + potential airway compromise

  • high dose steroids + airway support

Superior Vena Cava Obstruction

  • stenting of veins to keep them patent (though usually resolves with treatment of malignancy)

Tumour Lysis Syndrome

  • Hyperhydration!!!
  • Allopurinol or Rasburicase can also be used

Long term treatment = chemotherapy or radiotherapy depending on stage

SE = tumour lysis syndrome, neutropenia, alopecia, sub-fertility

(Life-long follow up)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the difference between foetal and adult haemoglobin?

A

Foetal haemoglobin = 2 alpha + 2 gamma subunits

  • this structure gives it a greater affinity to O2 than adult Hb
  • allows foetal Hb to steal O2 away from mothers Hb when nearby in the placenta
  • foetal Hb requires a lower partial pressure of O2 to fill up with O2 compared to adult Hb

Adult haemoglobin = 2 allpha + 2 beta subunits

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is sickle cell anaemia?

A
  • foetal Hb (2a+2g) is replaced by adult Hb (2a+2b) at around 6 weeks of age
  • patients with sickle cell disease have an abnormal variant called Haemoglobin S (HbS)
  • It is an autosomal recessive condition where there is an abnormal gene for Beta-Globin on Chromosome11
    • 2 copies of the gene results in sickle shaped RBCs
    • this structure makes RBC fragile and easily destroyed = haemolytic anaemia
    • patients are prone to various sickle cell crises
  • Patients with one copy of the gene = sickle-cell trait
    • usually asymptomatic
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the selective advantage of having sickle cell gene?

A

Having one copy of the sickle cell gene (sickle-cell trait) reduces the severity of malaria.

Therefore there is a selective advantage to having the sickle cell gene in areas of malaria eg. Africa, India, Middle east, Caribbean

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

How is sickle cell disease diagnosed?

A
  • at risk women are tested during pregnancy
  • it is also tested for at Newborn Screening Heel Prick Test at 5 days of age
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What are some common complications of sickle cell disease you need to be aware of?

A
  • anaemia
  • increased risk of infection
  • stroke (sickled cells can block blood flow to areas eg. brain)
  • Avascular necrosis in large joints eg. hip
  • Pulmonary hypertension
  • Painful and persistent penile erection = priapism
  • Chronic Kidney Disease
  • Sickle Cell Crises
  • Acute Chest Syndrome (a new radiodensity on CXR + fever/resp symptoms)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is a vaso-occlusive crisis in sickle cell anaemia?

A

Vaso-occlusive Crisis

  • when sickled RBCs clog capillaries and cause distal ischaemia
  • associated with dehydration + raised haematocrit
  • symptoms = pain, fever, infection symptoms if it is an infection triggering it
  • can cause Priapism in men (blood trapped in penis = painful and persistent erection)
    • treat with aspiration of blood
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is splenic sequestration crisis in sickle cell anaemia?

A
  • When RBCs block blood flow within the spleen
  • results in an acutely enlarged + painful spleen
  • pooling of blood in the spleen can cause severe anaemia + circulatory collapse (hypovolaemic shock)
  • Management = supportive, blood transfusions, fluid resus
    • Splenectomy is used in recurrent crises
    • recurrent crises can lead to splenic infarction = susceptibility to infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is an aplastic crisis in sickle cell anaemia?

A
  • temporary loss of the creation of new blood cells
  • Most commonly triggered by Parovirus B19 infection
  • Leads to anaemia
  • Management = blood transfusions (usually resolves spontaneously within a week)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is acute chest syndrome in sickle cell anaemia?

A

Diagnosis = fever/ resp symptoms + new infiltrates on CXR

  • can be due to infection (eg. pneumonia, bronchiolitis) or non-infective causes (eg. pulmonary vaso-occlusion or fat emboli)
  • a medical emergency!!!
  • Management:
    • antibiotics / antivirals for infection
    • blood transfusions for anaemia
    • incentive spirometry to encourage effective and deep breathing
    • artificial ventilation with Non-invasive ventilation or intubation
24
Q

What are some common causes of anaemia in infancy?

A
  • physiologic anaemia of infancy
  • Anaemia of prematurity
  • Blood loss
  • Haemolysis
    • haemolytic disease of the newborn (ABO or Rhesus incompatibility)
    • Hereditary spherocytosis
    • G6PD deficiency
  • Twin-Twin transfusion
25
Q

What is physiologic anaemia of infancy?

A
  • a normal dip in Hb around 6-9 weeks of age
  • high O2 delivery to tissues caused by the high Hb levels at birth cause a negative feedback
  • therefore production of erythropoietin by the kidneys is suppressed
  • therefore production of Hb by the bone marrow is suppressed
26
Q

Why are premature neonates more likely to become significantly anaemic in the first few weeks of life compared with term infants?

A
  • less time in utero receiving iron from mother
  • RBC creation cannot keep up with the rapid growth in the first few weeks
  • Reduced erythropoietin levels
  • Blood tests remove a significant proportion of their circulating vol
27
Q

What is haemolytic disease of the newborn?

A
  • Incompatibility between the rhesus antigens on the surface of the RBC of the mother and foetus.
  • When a Rhesus D Negative woman becomes pregnant with a Rhesus D Positive foetus (has the rhesus D antigen)…
    • foetal blood is likely to enter her bloodstream
    • mothers immune system produces antibodies to the Rhesus D Antigen on the foetal RBCs
    • Mother has now become sensitised to Rhesus D Antigens
    • It will rarely cause problems for this first pregnancy unless sensitisation happens early on eg. antepartum haemorrhage
  • If the next pregnancy is another Rhesus D Positive baby, the mothers anti-D Antibodies can cross the placenta into the foetus
    • the antibodies cause haemolysis of the foetal RBCs = anaemia + high bilirubin levels
    • This is known as haemolytic disease of the newborn

Check for immune haemolytic anaemia using a Direct Coombs Test (DCT)

28
Q

What are some more common causes of anaemia in older children?

A

Common causes:

  • iron deficiency anaemia (secondary to dietary insufficiency)
  • blood loss (eg. menstruation in older girls)

Less common causes:

  • Leukaemia
  • Thalassaemia
  • Sickle cell anaemia
  • Hereditary spherocytosis
  • Hereditary eliptocytosis
  • Sideroblastic anaemia
  • Helminth infection, with roundworms, hookworms or whipworms (common in poverty)
    • cause blood loss –> chronic anaemia + iron def
    • Treatment = single dose of albendazole or mebendazole
29
Q

What are some causes of microcytic anaemia?

A

TAILS

T - Thalassaemia

A - Anaemia of chronic disease

I - Iron deficiency anaemia

L - Lead poisoning

S - Sideroblastic anaemia

30
Q

What are some causes of normocytic anaemia?

A

3As and 2Hs

A - Acute blood loss
A - Anaemia of chronic disease
A - Aplastic anaemia

H - Haemolytic anaemia
H - Hypothyroidism

31
Q

What are some causes of megaloblastic macrocytic anaemia?

A

Megaloblastic = due to impaired DNA synthesis preventing the cell from dividing normally (results in a large abnormal cell)

  • B12 deficiency
  • Folate deficiency
32
Q

What are some causes of normoblastic macrocytic anaemia?

A
  • alcohol
  • reticulocytosis (either from haemolytic anaemia or blood loss)
  • hypothyroidism
  • liver disease
  • drugs like azathioprine
33
Q

What are some generic signs and symptoms of anaemia?

A

Symptoms:

  • tiredness
  • SOB
  • headaches
  • dizziness
  • palpitations

Signs:

  • pale skin
  • conjunctival pallor
  • tachycardia
  • raised resp rate
34
Q

What are some specific signs and symptoms of iron deficiency anaemia?

A

Symptoms:

  • Pica (dietary cravings for abnormal things like dirt)
  • Hair loss

Signs:

  • Koilonychia (spoon shaped nails)
  • Angular chelitis (inflammation of one or both corners of the mouth)
  • Atrophic glossitis (smooth tongue due to atrophy of papillae)
  • Brittle hair and nails
35
Q

What specific sign can occur in thalassaemia?

A

Bone deformities

36
Q

How would you investigate anaemia?

A
  • FBC for Hb and MCV
    Blood film
  • Ferritin
  • B12 and folate
  • Bilirubin (raised in haemolysis)
  • Direct Coombs Test (autoimmune haemolytic anaemia)
  • Haemoglobin electrophoresis (haemoglobinopathies)
  • Reticulocyte count (high when there is more active production of RBC to replace lost cells. generally indicative of anaemia due to haemolysis or blood loss)
    *
37
Q

What are some ways in which someone can beocme iron deficient?

A
  • dietary insufficiency (most common cause in children)
  • Loss of iron eg. heavy menstruation
  • Medications that reduce stomach acid (eg. PPIs lansoprazole or omeprazole)
    • acid from the stomach keeps iron in soluble ferrous (fe2+) form to eb absorbed.
    • when there is less acid, it changes to insoluble ferric (fe3+) form
  • Conditions that cause inflammation of the duodenum or jejunum resulting in inadequate absorption of iron
    • Eg. Coeliac disease, Crohns disease
38
Q

How would you test for iron deficiency?

A
  • Total Iron Binding Capacity (increases in iron deficiency)
  • Transferrin (increases in iron deficiency)
  • Serum Iron (not useful as it varies throughout the day. higher in the morning and after eating iron heavy meals)
  • Ferritin (low levels in the blood indicate iron deficiency)
    • however high levels can be the result of inflammation, not iron overload
    • someone with normal ferritin can be iron deficient (esp if they have another reason for ferritin to be high eg. an infection)

Iron supplements or acute liver damage can increase all these values and appear like the patient is iron overloaded

39
Q

How would you manage iron deficiency?

A
  • input from dietician if dietary deficiency
  • supplent with ferrous sulphate or ferrous fumarate
    • oral iron can cause constipation + black stools
    • not suitable where malabsorption is the cause of anaemia
  • Blood transfusions sometimes necessary
40
Q

What is Idiopathic Thrombocytic Purpura?

A
  • a condition characterised by spontaneous (idiopathic) thrombocytopaenia (low platelets)
  • causes a purpuric rash (non-blanching rash)
  • caused by a Type II hypersensitivity reaction (antibodies are produced that target and destroy platelets)
  • either spontaneous or can be triggered by eg. viral infection
41
Q

How does ITP present?

A
  • usually in kids under 10 years old
  • recent hx of viral illness

onset of symptoms over 24-48 hours:

  • bleeding eg. from gums, epistaxis, menorrhagia
  • bruising
  • petechial (1mm pin-prick spots of bleeding under the skin) or purpuric (larger 3-10mm spots of bleeding under the skin) rash
    • when a larger area of blood is collected (>10mm) this is an ecchymoses
    • All these lesions are non-blanching
42
Q

How would you manage ITP?

A

Treatment = in active bleeding or severe thrombocytpaenia (platelets<10)

  • Prednisolone
  • IV immunoglobulins
  • Blood transfusions if needed
  • Platelet transfusions are a temporary fix
    • the antibodies will soon begin destroying these new platelets too
  • Education:
    • avoid contact sports
    • avoid IM injections or procedures like LP
    • avoid NSAIDs, aspirin, blood thinning meds
    • advice on managing nosebleeds
    • seek help after any injury that could cause internal bleeding eg. a car accident or head injury
43
Q

How woudl you confirm ITP?

A

Confirm diagnosis by…

  • urgent FBC and platelet count (other values should be normal)
  • Exclude other causes of low platelets eg. heparin induced thrombocytopaenia, leukaemia
44
Q

What are thalassaemias?

A

A genetic defect in the protein chains that make up haemoglobin.

  • Normal hb is 2a+2b
  • defects in the alpha globin chains = alpha thalassaemia
  • defects in the beta globin chains = beta thalassaemia
  • both conditions are autosomal recessive
  • Thalassaemia patients have fragile RBC that easily break down and are sieved by the spleen = splenomegaly
  • Iron overload can occur due to faulty RBCs, recurrent transfusions and increased absorption of iron in the gut in response to anaemia
    • manage this by limiting transfusions and performing iron chelation
  • The Bone marrow also expands to produce extra RBC to replace destroyed ones and compensate for the chronic anaemia
    • = susceptibility to fractures + prominent features eg. pronounced forehead and malar eminences (cheekbones)
45
Q

How would you diagnose a thalassaemia?

A
  • FBC = microcytic anaemia
  • Haemoglobin electrophoresis to diagnose globin abnormalities
  • DNA testing to look for genetic abnormalities
  • Pregnant women are offered a thalassaemia screening test at booking appointment
46
Q

What is alpha thalassaemia and how would you manage it?

A

defects on chromosome 16 causing defects in alpha globin chains

Management:

  • monitoring FBC
  • monitoring for complications
  • blood transfusions
  • splenectomy can be performed
  • bone marrow transplant can be curative
47
Q

What are the different kinds of beta thalassaemia and how would you manage them?

A

Beta thalassaemia is caused by defects in beta globin chains (defects on chromosome 11)

Thalassaemia minor = carry 1 abnormally functioning beta globin gene and 1 normal gene

  • mild microcytic anaemia
  • monitor, dont usually require active treatment

Thalassaemia Intermedia = carry 2 abnormally b globin genes (2 defective genes OR 1 defective gene and 1 deletion gene)

  • more significant microcytic anaemia
  • monitoring
  • occasional blood transfusions
  • iron chelation to prevent iron overload from transfusions

Thalassaemia Major = homozygous for deletion genes

  • severe microcytic anaemia
  • failure to thrive in early childhood
  • bone deformities
  • splenomegaly
  • Management = regular transfusions, iron chelation, splenectomy
    • bone marrow transplant can be curative
48
Q

What is hereditary spherocytosis?

A

RBCs are sphere shaped, making them easily destroyed when passing through the spleen.

Autosomal dominant, common in northern europeans

49
Q

How would hereditary spherocytosis present?

A
  • jaundice
  • anaemia
  • gallstones
  • splenomegaly
  • episodes of haemolytic crisis triggered by infections (presents with increased haemolysis, anaemia, jaundice)
  • can develop aplastic crisis (presents with increased haemolysis, anaemia, jaundice)
    • lack of normal response from bone marrow of creating new RBC in response to haemolysis (increased reticulocytes)
    • therefore in aplastic crisis, you do not get the reticulocyte response.
    • Aplastic crisis is often triggered by parvovirus infection
50
Q

How would you diagnose hereditary spherocytosis?

A

Diagnosis

  • fhx and clinical features
  • spherocytes on blood film
  • mean corpuscular haemoglobin conc (MCHC) is raised on FBC
  • reticulocytes are raised due to rapid turnover of RBCs
51
Q

How would you manage hereditary spherocytosis?

A
  • folate supplementation and splenectomy
  • Cholecystectomy (removal of gallbladder) can be required if gallstones are a problem
  • Transfusions can be required in acute crises
52
Q

What is hereditary elliptocytosis?

A

similar to hereditary spherocytosis but the RBCs are ellipse shaped.

Autosomal dominant

(similar presentation and management)

53
Q

What is G6PD deficiency?

A

Defect in the G6PD enzyme normally found in all cells in the body.

  • it is responsible for protecting cells from damage by reactive oxygen species (ROS)
  • ROS are produced in normal cell metabolism, but produced in higher quantities during stress on the cell
  • G6PD deficiency in RBCs leads to more haemolysis
  • In periods of increased stress and increased ROS, G6PD deficiency can result in acute haemolytic anaemia.

Inherited in an X linked recessive pattern (usually affects males).

Causes crises that are triggered by infections, medications or fava beans (broad beans)

54
Q

How does G6PD deficiency present and how is it diagnosed?

A
  • neonatal jaundice
  • anaemia
  • intermittent jaundice (in response to triggers)
  • gallstones
  • splenomegaly
  • hx of antimalarial medications, eating broad beans, developing an infection

Investigations:

  • Heinz bodies on blood film
  • G6PD enzyme assay to diagnose
55
Q

How would you manage G6PD deficiency?

A

avoid triggers:

  • Fava beans
  • medications:
    • primaquine (antimalarial)
    • ciprofloxacin
    • nitrofurantoin
    • trimethoprim
    • sulfonylureas (eg. gliclazide)
    • sulfasalazine and other sulphonamide drugs

4th Year (17 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions