Haematology/oncology Flashcards
What level of haemoglobin is equivalent to anaemia in a child?
<11g/dL (from birth until puberty)
Most common cause of iron deficiency anaemia?
Inadequate intake
Early signs of iron deficiency anaemia?
Dyspnoea and increasing tiredness
Slow/poor feeding
Pallor (pale conjunctiva, palmar creased, skin)
What is Pica?
This is a late sign of iron deficiency anaemia - it is inappropriate eating e.g. chalk, gravel, sand etc.
What blood tests would be performed and what would they show in iron deficiency anaemia?
FBC - decreased Hb, MCV, MCH and MCHC, increased platelet count
(WCC - eosinophils++ in hookworm infection)
Serum ferratin - reduced
Serum iron - <6-7 g/dl
Increased total iron binding capacity
Blood film = Microcytic and hypochromic RBC
Dietary advice given after diagnosis of iron deficiency anaemia?
Milk:
Breat milk - low Fe content but good absorption (50%)
Cows milk - high Fe content but low absorption (20%) - avoid if <1yr
Infant formula - supplement iron to increase intake
Eat vitamin C rich foods - fruit and dark green veg
Eat beef, lamb, liver, kidney (high in iron)
Further management of iron deficiency anaemia?
ferrous sulphate salt - 5mg/kg/day in 2-3 doses (do not exceed 200mg/day)
Continue for 3 months until Hb normalises - if not screen for thalassaemia
What type of brain tumour is most common in children?
Astrocytoma (40%)
What are the different types of brain tumour seen in children?
Astrocytoma = vary from benign to highly malignant (glioblastoma multiforme)
Medulloblastoma (20%) - form in the midline of the posterior fossa. May seed through CNS via CSF –> 20% have soinal metastases at presentation
Ependyoma (8%) - mostly in the posterior fossa and act like a medulloblastoma
Brainstem glioma (6%)
Craniopharyngoma - remnant of Rathke’s Pouch - not truly malgnnat but slowly invasive
Clinical presentation of a brain tumour?
Raised ICP +/- focal neurological signs
Spinal tumour mets –> back pain, paraesthesia, (chronic back pain should always be investigated by MRi in children)
Investigations and management for brain tumours
MRI head = gold standard
Surgery +/- radio/chemotherapy - primarily to reduce hydrocephalus, biopsy and maximal resection of tumour
What type of disorder is haemophilia?
An X-linked autosomal recessive of the clotting factors - 1/10,000 males
Haemophilia A = FVIII
Haemophilia B = FIX
Symptoms of Haemophilia?
Easily bruising, nose/gum bleeds
Bleeding in joints/muscle
Prolonged bleeding after trauma
Intracranial bleeds
Signs - haematuria, multiple bruises
Investigations for Haemophilia?
Bloods: FBC, U+E’s, LFTs, TFTs
CT head for intracranial bleeds
Clotting tests:
APTT = increased (intrinsic part of the clotting cascade)
PT/INR = normal
Thrombin time = normal
FVIII:C ratio = reduced (indicates severity)
vWF - important to differentiate from von Willebrand’s disease (similar results in clotting tests - not as severe)
Management of haemophilia?
Replace missing clotting factors when bleeds occur
Prophylactic clotting factors in severe disease
Desmopressin increases levels of vWF and FVIII:C therapeutically in haemophilia A (and von Willebrand disease)
Transexamic acid (TXA) for mouth bleeds
Lifestyle - avoid IM injections, contact sports, aspirin, NSAIDS
What is HSP?
Small vessel vasculitis often preceded by URTI (strep) - immune complexes IgG and IgA attacked by compliment cascade
Clinical features of HSP?
- Palpable papular non-blanching purpuric rash - usually buttocks and lower legs (trunk spared)
- Arthralgia/arthritis
- Abdominal pain (colicky)
Other features: Fever, UTI, oedema, melena,
Management of HSP?
Self-limiting - supportive
NSAIDS - for arthritic pain
Corticosteroids for arthritis/abdo pain
If severe - high dose steroids +/- cyclophosphamide
Complications of HSP?
Nephritic syndrome
Intussusception/appendicitis –> bowel perforation
What is the most common type of leukaemia in children?
Acute lymphoblastic leukemia (ALL) - 80% - peaks 2-5yrs
Clinical presentation of ALL?
General - anorexia, malaise
Bone marrow - anaemia (pallor, lethargy), infections (neutropenia), thrombocytopenia (bruising, rash) and bone pain
Reticulo-endothelial infiltration - hepatospenomegaly, mediastinal lymphadenopathy
Symptoms typical of a relapse?
Organ filtration
- -> CNS = headaches, nausea, vomiting
- -> Testes = enlargement
Diagnosis of leukaemia?
Bloods - FBC (low WCC, Hb, platelets +/- leukaemic blast cells)
Clotting sceen, ESR, U+Es
Bone marrow - leukaemic blast cells and leucocytosis = diagnostic, gives useful diagnostic info
What management options are there before trying to reduce remission of leukaemia?
Correct underlying electrolyte/cellular inbalances i.e. blood transfusion for anaemia
Preventative measures:
Allopurinol - protects kidneys from rapid cell lysis
Prophylactic Co-trimoxozole to prevent pneumocystis carinii pneumonia
Treatment course for ALL?
Induced remission = combinant chemotherapy - 95% success rate
Intensification - block of intense chemo to consolidate remission
CNS - intrathecal chemo (systemic chemo in circulation crosses into CNS poorly)
Continuing therapy - 3yrs (but low modest intensity)
Relapses? - High dose chemo and total body irradiation
Bone marrow transplantation
What is the prognosis of leukaemia?
~80% survival
Poorer if: male, high WCC at diagnosis, high minimal residual disease (MRD), age <2 or >10yrs
When would a headache be worst in the day in raised ICP?
In the morning (vomiting also worse first thing in the morning)
What type of lymphoma is the most common?
Non-Hodgkins lymphoma (80%)
How do lymphomas usually present? How else might they present?
Usually present with painless lymphadenopathy that is much firmer than it’s benign counterpart
can cause obstruction in airways or SVC
Can also develop ‘B’ symptoms - fever, itching, night sweats, weight loss
Investigations into lymphomas?
Lymph node biopsy
Radiological assessment for staging
Bone marrow biopsy
PET scans to monitor treatment response when giving combinant chemo (+/- radiotherapy) in Hodgkins
Pathophysiology of sickle cell disease?
HbA–> HbS
Polymerises to rigid tubular spiral bodies
Decreased RBC lifespan +/- trapped in microcirculation –>vaso-oclusive crisis
How does vaso-oclusive crisis present clinically?
Pain+++ most commonly in the hands and back
Iachemia +/- necrosis/end organ damage
Can lead to acute chest syndrome (SOB, hypoxia, yellow sputum)
What are the different types of sickle cell disease?
HbSS = true form (two copies of disease) - 50% mortality by 40 yrs
HbSC = One copy of HbS + another abnormal Hb (progressive retinopathy of adolescence +/- osteoporosis of hips/shoulders)
Sickle trait = carrier
Diagnosis of sickle cell disease?
Guthrie (heel prick test) at 5 days = screening - will develop symtpoms at 6 months
Hb will usually be between 6-10g/dL
Management of sickle cell disease?
Full imunisation is a priority
Penicillin V as prophylaxis against encapsulated pathogens
Folic acid daily
Lifestyle - avoid dehydration, extreme cold
Complications of sickle cell disease?
Encapsulate organism infection: Pneumococcal sepsis (>3yrs most at risk) Aplastic crisis (after parvovirus B19)
Vaso-oclusive crisis Avascualr necrosis (hips and humerus) Sequestrian crisis - sickle cells in spleen - can lead to infarcts (abdo pain, circulatory collapse) Retinopathy Acute chest syndrome (SOB, hypoxia, yellow sputum) Priaprism (painful erection) Stroke (5-10yr olds) Hepatic pain
Management of sickle cell disease crisis?
O2
IV fluids - aim for 150% of maintenance
Abx if infection (cephalosporin)
analgesia
Where does the neural crest tissue arise from in neuroblastoma?
Adrenal medulla or the sympathetic nervous system
Who does neuroblastoma usually affect?
Children under five
What are common signs that the disease has progressed metastatically?
Bone marrow suppression –> malaise, pallor and weight loss
Bone pain
Hepatomegaly
What might be found upon urinalysis in neuroblastoma? What other investigations should be done?
Catecholamines
Bloods
MRI/CT
Positive I-MIBG radioisotope scan +/- bone scan
Tumour and bone marrow biopsy
In localised neuroblastoma what is the management? What further management is required in metastatic disease?
Surgery is the only management needed in localised disease
Chemo/radiotherapy are used in metastatic followed by immunotherapy (e.g. retenoic acid) for maintenance
Why do haemoglobinopathies that affect the beta Hb chain (B thalassaemia and sickle cell) only present after 3-6 months
This is when the HbF present at birth is replaced by HbA
Symptoms of B-thalassaemia?
Severe anaemia at 3-6 months
FTT
Jaundice
Why might heterozygous B-thalassamia or minor a-thalassaemia be confused with iron deficiency?
It can result in hypochromic RBCs
How might a foetus survive the pregnancy if it has the most severe form of a-thalassaemia?
Intra-uterine blood exchange transfusions
What is Hb barts and how is it diagnosed?
Most severe form of a-thalassaemia (deletion mutation of all 4 a-chains) - results in foetal hydrops (severe odema and jaundice)
Can be detected mid trimester - Hb electrophoresis or Hb HPLC
Management of symptomatic thalassaemia (i.e. results in severe anaemia)
Monthly exchange blood transfusions from 3-6 months of age (in utero and from birth in Hb barts)
+ iron chelation tehrapy (desferrioxamine SC) after 2-3 years to prevent iron over load
When do 80% of wilms tumours present?
before the age of 5
V. rare to present after 10 yrs
Investigations into wilms tumour?
CT/MRI will show mass distorting usual architecture of kidney and may show any metastases
Management of Wilm’s tumour?
- Initial chemo
- Neohrectomy + histology for staging
- Chemotherapy +/- radiotherapy (reserved fro severe disease)
80% cure rate (60% if metastatic)
