3. Haematology + Oncology Flashcards
What risk factors are there for developing iron deficiency anaemia?
Pregnancy
-Preterm
-LBW infants
-Multiple births
Nutrition
-After exclusive breastfeeding for >6 months
-Delayed introduction of iron-containing solids
-Excessive cow’s milk
-Poverty
-Strict vegans
Adolescent females
-Growth spurt
-Menstruation
Malabsorption
-Coeliac disease
-IBD
Blood loss
-Meckel’s diverticulum
-Oesophagitis
-Cysts
-Tumours
-NSAIDs
Intestinal parasites eg hookworm
How does iron deficiency anaemia present?
-Most cases are subclinical
-Toddlers can tolerate surprisingly low Hbs - adapt to their anaemia
-If severe - pallor, lethargy, poor feeding, SOB, irritability, reduced cognition and psychomotor performance
-Rare = earring unusual items
How is anaemia diagnosed?
NB anaemia = a sign not a disease, always look for underlying cause
-FBC - Hb, MCV, platelets (often raised)
-Blood film
-Serum ferritin (iron stores, often low)
How should you treat anaemia?
-5mg/kg elemental iron/day - oral ferrous salt
-Response seen in 5-10 days (continue for 3 months after Hb has normalised
-Dietary counselling
How do brain tumours normally present?
NB most occur in brainstem / cerebellum
Raised ICP / neurological signs
-Headache
-N+V
-Blurred vision
-Squint (CN VI palsy)
-Ataxia
-Clumsiness
-Head tilt
-Endocrine dysfunction
What are low grade gliomas and what is their prognosis?
-45% of CNS tumours
-Many are benign tumours
-Commonly occur in cerebellum and optic pathways
-Prognosis depends on site eg brainstem = high mortality, optic pathway = high morbidity
-50% of optic pathway low grade gliomas are neurofibromatosis type 1 tumours
What are high grade gliomas and what are their prognosis?
-10% of CNS tumours
-Predominantly occur in older children
-Difficult to manage as complete resection is essential for a good outcome but is difficult to achieve
-RT normally mainstay of treatment
What is a diffuse brainstem glioma?
-Glioma in pons - usually high grade and inoperable
-RT mainstay of treatment
-Median survival <1yr
What is a primitive neuroectodermal tumour and what is their prognosis?
-25% of CNS tumours
-Majority occur in the cerebellum (=medulloblastoma)
-Peak incidence at 2-6yrs
-Tumour mets in spine in 20% at diagnosis
-70% of localised cases can be cited, but significant morbidity from RT
What is an ependyma and what is its prognosis?
-10% of CNS tumours
-Periventricular sites
-Obstructive hydrocephalus at presentation
-10% –> spine mets
-Surgical excision + RT
->70% survival if complete excision
What are CNS germ cell tumours?
-5% of CNS tumours
-Secreting tumours - raised markers
-Surgery for teratomas, CT+RT for others
What is a craniopharyngioma?
-10% of CNS tumours
-Arise from squamous remnant of Rathke’s pouch
-Locally invasive
-Visual field loss, pituitary dysfunction
-Surgery + RT
What is a retinoblastoma and what is its prognosis?
-3% of all tumours
-Present with squint / visual disturbances and abnormal light reflex
-Surgery, chemo, focal therapy
-90% 5YSR
What is causes haemophilia and what is its inheritance pattern?
-X-linked recessive inheritance
-H-A = defective production of Factor VIII
-H-B = deficiency in Factor IX (less common)
-Carrier females
How does the severity of Haemophilia vary?
Mild disease = rarely bleed, prophylaxis needed for surgery
Moderate = bleeding secondary to trauma, requires FVIII concentrate when bleeding occurs
Severe = spontaneous bleeds, requires prophylactic FVIII concentrate
What are haemarthroses?
-Bleeding into joints
-Joint becomes painful, swollen, tender and warm
-Severe limitation of movement
-May be unable to weight bear
How would you investigate haemophilia?
-APTT (increased), FVII
-PT (normal)
-Plasma FVIII and IX assay
-Cranial CT if any suspicion of bleed
-USS if ?haemarthroses
How would you manage haemophilia?
-Prophylaxis = regular otocog / nonacog alpha every 48h (A) / twice a week (B)
-On demand = desmopressin + otocog (A) / nonacog alpha (B)
-Prior to major surgery = FVIII/IX + stop NSAIDs
-Prior to minor surgery / persistent bleeds = desmopressin + factor + aminocaproic acid
-Avoid IM injections
-Educate family on ice, rest, compression, elevation
What is Henoch Schonlein purpura?
-Small vessel vasculitis - associated with IgA
-Commonly follows an URTI / exposure to drug / allergen
-Most commonly affects pre-pubertal boys
How does Henoch Schonlein purpura present?
Triad - GI + skin rash + polyarthritis
GI:
-Colicky abdo pain (most common)
-Malaena
-Haematemesis
-Intussusception
-Perforation
-Appendicitis
Skin rash:
-Palpable, papular, purpuric rash
-Starts on buttocks –> lower legs, flexor surfaces
-Can lead to oedema
Polyarthritis:
-Typically short lived, affects large joints
NB associated with glomerulonephritis in 50%
How would you investigate Henoch Schonlein purpura?
-Diagnosis is clinical but must exclude other causes of purpura by confirming normal platelet and coagulation studies
-FBC
-U+Es
-BP
-Urine dip (protein/haematuria)
-Full renal investigation with biopsy (if evidence of renal involvement)
-Abdo investigations as per symptoms
How would you treat Henoch Schonlein purpura?
-Most are self-resolving within 6 weeks
-NSAIDs for arthralgia
-Steroids for any abdominal pain accompanied by N+V
What are the differences between different forms of leukaemia?
-Acute lymphoblastic leukaemia (80%) –> malignant proliferation of pre-B or T-cell lymphoid precursors in bone marrow
-Acute myeloid leukaemia –> granulocytic / monocytic cells
-ALL has slightly better prognosis
-Cause of both is unknown
How does leukaemia present?
-Malaise
-Anorexia
-Anaemia - lethargy and pallor
-Neutropenia - frequent / severe infections
-Bruising
-Bone pain
-Lymphadenopathy / splenomegaly / hepatomegaly
-Testicular swelling
