Haematology/oncology Flashcards by Ben Cain

What level of haemoglobin is equivalent to anaemia in a child?

<11g/dL (from birth until puberty)

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Most common cause of iron deficiency anaemia?

Inadequate intake

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Early signs of iron deficiency anaemia?

Dyspnoea and increasing tiredness
Slow/poor feeding
Pallor (pale conjunctiva, palmar creased, skin)

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What is Pica?

This is a late sign of iron deficiency anaemia - it is inappropriate eating e.g. chalk, gravel, sand etc.

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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

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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)

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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

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What type of brain tumour is most common in children?

Astrocytoma (40%)

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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

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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)

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Investigations and management for brain tumours

MRI head = gold standard

Surgery +/- radio/chemotherapy - primarily to reduce hydrocephalus, biopsy and maximal resection of tumour

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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

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Symptoms of Haemophilia?

Easily bruising, nose/gum bleeds
Bleeding in joints/muscle
Prolonged bleeding after trauma
Intracranial bleeds

Signs - haematuria, multiple bruises

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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)

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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

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What is HSP?

Small vessel vasculitis often preceded by URTI (strep) - immune complexes IgG and IgA attacked by compliment cascade

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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,

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Management of HSP?

Self-limiting - supportive
NSAIDS - for arthritic pain
Corticosteroids for arthritis/abdo pain

If severe - high dose steroids +/- cyclophosphamide

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Complications of HSP?

Nephritic syndrome

Intussusception/appendicitis –> bowel perforation

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What is the most common type of leukaemia in children?

Acute lymphoblastic leukemia (ALL) - 80% - peaks 2-5yrs

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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?

1. Initial chemo 2. Neohrectomy + histology for staging 3. Chemotherapy +/- radiotherapy (reserved fro severe disease) 80% cure rate (60% if metastatic)