Haem + onco Flashcards

1
Q

Which type of leukaemia is the one which most commonly and almost only affects children?

A

Acute lymphoblastic leukaemia (peaks between ages 2-5)

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2
Q

What investigations should be conducted in someone with suspected ALL

A
  • FBC: shows pancytopenia or anaemia and thrombocyotpenia
  • blood film- blast cells present
  • chest xray- to check for mediastinal mass
  • bone marrow aspirate: to see extent of infiltration
  • lumbar puncture for CNS involvement
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3
Q

How does ALL present?

A
  • anaemia
  • thrombocyopenia - easy bruising and bleeding
  • leukopenia: fevers/ infections
  • bone pain: increased pressure from hyperplastic marrow
  • weight loss and malaise
  • CNS involvement: headaches, seizures
  • high WBC may lead to lymphadenopathy
  • hepatosplenomegaly
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4
Q

Give 1 major risk factor for ALL

A
  • trisomy 21
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5
Q

How is ALL managed?

A
  • resus and stabilisation (may need hyperhydration if hyperviscosity due to high WBC)
  • Aggressive IV, oral and intrathecal chemo
  • supportive blood products and prophylactic anti- fungal
  • maintenance therapy for 2 yrs for girls and 3 yrs for boys
  • some need allogenic BMT
  • 90% survive
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6
Q

How may lymphoma present?

A
  • non tender lymphadenopathy- may be intraabdominal or mediastinal so not always palpable
  • weight loss
  • night sweats
  • fevers
  • lethargy, anorexia
  • may have cough/ wheeze/ SVCO if mediastinal mass
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7
Q

How should suspected lymphoma be investigated?

A
  • FBC- infection differential
  • U&E- tumour lysis syndrome
  • LDH levels are usually elevated
  • USS- identify other nodes and assist biopsy
  • Chest X- ray- mediastinal node involvement
  • Full body CT- determine the extent of the disease
  • Lymph node biopsy
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8
Q

give 2 riskfactors for lymphoma

A
  • EBV
  • immunosurpressed
  • pts on cancer treatment
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9
Q

Describe the staging of lymphoma

A
  • STAGE 1: single group of lymph nodes/ single organ
  • STAGE 2: 2 or more groups of lymph nodes/ organs same side of the diaphragm
  • STAGE 3: lymph nodes or organs on both sides of the diaphragm
  • STAGE 4: diffuse involvement of lymph nodes and organs, e.g. liver and bones
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10
Q

how is lymphoma managed?

A
  • Mediastinal mass with potential airway compromise- high dose steroids and airway support
  • Superior vena cava obstruction (SVCO)- stenting of veins to keep patent
  • Tumour lysis syndrome (causes release of large amounts of phosphorus, potassium and calcium- potential kidney damage) - hyperhydration + allopurinol
  • Long term treatment is with chemotherapy and radiotherapy
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11
Q

How does a nephroblastoma/ wilms tumour present?

A
  • median age is 3.5
  • incidental abdo mass finding (usually unilateral but may be bilateral)
  • abdominal swelling
  • abdo pain
  • fever
  • haematuria
  • HTN
  • may present late with signs of compression of other intra- abdominal structures
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12
Q

Describe the staging of wilms tumours?

A
  • 1: tumour confined to the kidney + can be completely removed in surgery
  • 2: tumour has begun to spread beyond the kidney, but can be removed in surgery
  • 3: tumour cannot be completely surgically resected because spread to lymph nodes
  • 4: Distant metastases- commonly the lungs
  • 5: Bilateral tumours
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13
Q

How are wilms tumours managed?

A
  • supportive: treat infections, ensure optimum nutrition and hydration
  • surgery: to preserve renal function and remove malignant tumour
  • chemo: to reduce malignant tissue before surgery
  • 85% are cured
  • long term need to take steps to protect remaining good kidney: good BP control, avoid contact sports
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14
Q

What is immune thrombocytopenia (ITP)

A
  • autoimmune disorder where platelet count is reduced
  • primary is where platelets are destroyed in isolation
  • secondary is due to other causes/ where other things also go wrong
  • most occur in children following viral infection or immunisation, is self limiting and they recover within 6-8 weeks
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15
Q

Give 5 secondary causes of ITP

A
  • autoimmune disorders: SLE, antiphospholipid antibody syndrome
  • infection: hep C, HIV, varicella zoster, h. pylori
  • meds
  • lymphoproliferative disorders
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16
Q

Give 5 symptoms of ITP

A
  • no symptoms
  • petechiae
  • bruising
  • nose bleeds
  • haematuria and GI bleeds (less common)
  • intracranial bleeds (rare)
17
Q

Give 3 differentials for ITP

A
  • aplastic anaemia
  • leukaemia
  • von willebrands
  • meningococcal septisaemia
  • NAI
  • DIC
  • HSP (rash on posterior legs and bum)
18
Q

How is ITP managed? (if not actively bleeding)

A
  • treatment based on symptoms rather than platelet count:
  • trauma prevention and advice to avoid contact sports
  • avoid aspiring and NSAIDS
  • safety net and can send home if not actively bleeding
19
Q

How should ITP be managed where there is active bleeding?

A
  • 1st line: pred + IV immunoglobulins
  • 2nd line: rituximab, high dose dexamethasone
  • tranaxamic acid
  • emergency platelet infusions
  • surgery: if source, also splenectomy if thats bleeding
20
Q

Give 5 common causes of iron deficiency anaemia in children

A
  • blood loss: NSAID use, hook worm, gastric or duodenal ulcer, angiodysplasia, menorrhagia, recurrent epistaxis, IBD
  • dietary inadequacy: vegetarian/ vegan, rapidly growing children
  • failure to absorb: tetracyclines/ quinolones, antacids and PPIs, Calcium supplements, coeliac, IBD etc
21
Q

Describe how anaemia may present

A
  • fatigue
  • SOB
  • palpitations
  • sore tongue/ taste disturbance
  • hair change/ loss
  • pruritis
  • headache
  • tinnitus
  • angina
  • pallor, kolionychia, angular chellitis, atrophic glossitis and tachycardia on exam
22
Q

How should anaemia be investigated in a child

A
  • FBC: shows a hypochromic microcytic anaemia- low MCH and low MCV (if iron deficiency)
  • Serum ferritin
  • Blood film: anisocytosis and poikilocytosis
  • Urine test
  • Screened for coeliac disease
23
Q

how is iron deficiency anaemia managed?

A
  • oral iron supplement
  • advice to increase uptake of iron rich foods (dark green veg, meat, apricots, prunes, raisins and iron fortified bread)
  • blood transfusions not necessary if due to dietary insufficnecy
  • IV iron if oral unsucessful
24
Q

Give 3 side effects of oral iron supplements

A
  • constipation
  • black stools
  • heartburn
  • nausea
  • epigastric pain
  • some stain teeth
  • diarrhoea
25
Q

Describe how response to iron supplementation should be monitored and managed?

A
  • check FBC 2-4 weeks after starting iron supplement (hb should increase by around 10g/l/ week
  • if there is response, check every 2-4 weeks to ensure they return to normal
  • when Hb normal, continue treatment for 3 months
  • then recheck iron every 3 months for a year and then recheck again after a further year
26
Q

List 4 causes of haemolytic anaemia in children?

A
  • sickle cell
  • thalassaemia
  • g6pd deficiency
  • hereditary spherocytosis
  • autoimmune
  • haemolytic uraemic syndrome
27
Q

When does B thalassaemia present compared to alpha?

A

Beta tends to present later- after 6 months when most of the HbF (a and y) present at birth has been replaced by HbA (a and B chains)

28
Q

What may precipitate a sickle cell crisis?

A

low oxygen
dehydration
cold

29
Q

Describe the clinical features of sickle cell disease?

A
  • anaemia: increased haemolysis-> haemolytic anaemia + jaundice
  • infection esp from encapsulated bacteria eg pneumoccci and H. influenzae: due to hyposplenism secondary to chronic microinfarction
  • painful vaso- occulsive crisis: hand-foot syndrome (dactylitis and swlling and pain in fingers or feet), AVN femoral head, actute chest syndrome
  • acute anaemia: sudden Hb drop due to infections, parovirus causing aplastic crises or sequestration
  • priaprism: need exchange transfusion to prevent fibrosis of copora cavernosa
  • splenomegaly: common in younger kids
30
Q

What long term problems are associated with sickle cell disease?

A
  • short stature and delayed puberty
  • stroke and cognigitve problems
  • adenotonsillar hypertrophy
  • cardiac enlargement: from chronic anaemia
  • heart failure: from uncorrected anaemia
  • renal dysfunction
  • pigment gall stones
  • leg ulcers
  • psychosocial problems
31
Q

How should sickle cell be managed? (acute crises and generally)

A
  • prophylaxis and vaccination from encapulsed organisms
  • folic acid supplement as increased demand from haemolysis
  • avoid vaso occulsive crises by minimising cold exposure, keeping well hydrated, avoiding excessive exercise and stress or hypoxia
  • acute crises managed w/ oral/ IV analgesia, oral/ IV hydration, abx if infection, oxygen if hypoxia. Exchange tranfusions indicated by acute chest syndrome, stroke and priaprism
  • hydroxycarbamide can be given if getting lots of vasocclusive crises/ acute chest syndromes. This increased HbF production. Bone marrow transplant can be offered for non responders or those thatve had a stroke- only possible if HLA identicle sibiling
32
Q

Describe the clinical features of thalassaemia

A
  • severe anaemia
  • faltering growth/ growth failure
  • extra medullar haematopoesis if not havin regular transfusions (hepatosplenomegaly and bone marrow expansion- maxillary overgrowth and skull bossing)
33
Q

How is B- thalassaemia managed?

A

Major:

  • fatal without regular (monthly) blood transfusions to keep Hb >100
  • give with iron chelating agents
  • bone marrow transplant from HLA matched sibling has 90-95% success rate and 5% mortality
34
Q

What is prognosis like for a- thalassaemia?

A

4 a- globin gene deletions = thalassaemia major or barts hydrops fatalis. Only survive if months intrauterine transfusions then monthly transfusions from birth
3 a- globin gene deletions= HbH disease- mild- mod anaemia, some pts are transfusion dependant.
1 or 2 gene deletions= a- thalassaemia trait, usually asymptomatic.

35
Q

Do does Von Willebrands differ from haemophilias in presentation?

A
  • VWD: mucous membrane bleeding (epistaxis/ menorrhagia) and skin (bruising) haemorrhage
  • Haemophilia tends to present w/ bleeding into muscles or joints
  • VWB tends to present in adolescence w/ menorrhagia, haemophillas will present earlier- w/ intracranial haemorrhage or bleeding after procedure in neonate period, or as toddler when starts to walk
36
Q

What will be abnormal in blood tests of haemophilia A and B?

A

APTT- this measures factors II,V, VIII (haemophillia A) , IX (Haemophilia B),X,XI, XII
- PT measures II,V, VII, X (so will be normal)

37
Q

How is haemophilia managed?

A
  • recombinant factor VIII (for A) or IX (B)
  • avoid IM injections, aspirin, NSAIDS
  • desmopressin can be used for mild haemophilia A as increases endogenous release of FVIII and vWF
38
Q

How is von willebrands managed?

A
  • Desmopressin if type 1 (less severe)
  • recombinant factor VII if mor severe
  • Avoid IM injections, aspirin and NSAIDS