Haem + onco Flashcards
Which type of leukaemia is the one which most commonly and almost only affects children?
Acute lymphoblastic leukaemia (peaks between ages 2-5)
What investigations should be conducted in someone with suspected ALL
- 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
How does ALL present?
- 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
Give 1 major risk factor for ALL
- trisomy 21
How is ALL managed?
- 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
How may lymphoma present?
- 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
How should suspected lymphoma be investigated?
- 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
give 2 riskfactors for lymphoma
- EBV
- immunosurpressed
- pts on cancer treatment
Describe the staging of lymphoma
- 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
how is lymphoma managed?
- 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
How does a nephroblastoma/ wilms tumour present?
- 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
Describe the staging of wilms tumours?
- 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
How are wilms tumours managed?
- 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
What is immune thrombocytopenia (ITP)
- 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
Give 5 secondary causes of ITP
- autoimmune disorders: SLE, antiphospholipid antibody syndrome
- infection: hep C, HIV, varicella zoster, h. pylori
- meds
- lymphoproliferative disorders
Give 5 symptoms of ITP
- no symptoms
- petechiae
- bruising
- nose bleeds
- haematuria and GI bleeds (less common)
- intracranial bleeds (rare)
Give 3 differentials for ITP
- aplastic anaemia
- leukaemia
- von willebrands
- meningococcal septisaemia
- NAI
- DIC
- HSP (rash on posterior legs and bum)
How is ITP managed? (if not actively bleeding)
- 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
How should ITP be managed where there is active bleeding?
- 1st line: pred + IV immunoglobulins
- 2nd line: rituximab, high dose dexamethasone
- tranaxamic acid
- emergency platelet infusions
- surgery: if source, also splenectomy if thats bleeding
Give 5 common causes of iron deficiency anaemia in children
- 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
Describe how anaemia may present
- fatigue
- SOB
- palpitations
- sore tongue/ taste disturbance
- hair change/ loss
- pruritis
- headache
- tinnitus
- angina
- pallor, kolionychia, angular chellitis, atrophic glossitis and tachycardia on exam
How should anaemia be investigated in a child
- 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
how is iron deficiency anaemia managed?
- 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
Give 3 side effects of oral iron supplements
- constipation
- black stools
- heartburn
- nausea
- epigastric pain
- some stain teeth
- diarrhoea
Describe how response to iron supplementation should be monitored and managed?
- 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
List 4 causes of haemolytic anaemia in children?
- sickle cell
- thalassaemia
- g6pd deficiency
- hereditary spherocytosis
- autoimmune
- haemolytic uraemic syndrome
When does B thalassaemia present compared to alpha?
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)
What may precipitate a sickle cell crisis?
low oxygen
dehydration
cold
Describe the clinical features of sickle cell disease?
- 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
What long term problems are associated with sickle cell disease?
- 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
How should sickle cell be managed? (acute crises and generally)
- 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
Describe the clinical features of thalassaemia
- severe anaemia
- faltering growth/ growth failure
- extra medullar haematopoesis if not havin regular transfusions (hepatosplenomegaly and bone marrow expansion- maxillary overgrowth and skull bossing)
How is B- thalassaemia managed?
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
What is prognosis like for a- thalassaemia?
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.
Do does Von Willebrands differ from haemophilias in presentation?
- 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
What will be abnormal in blood tests of haemophilia A and B?
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)
How is haemophilia managed?
- 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
How is von willebrands managed?
- Desmopressin if type 1 (less severe)
- recombinant factor VII if mor severe
- Avoid IM injections, aspirin and NSAIDS
