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Y4 Paeds CCs > 3. Haematology + Oncology > Flashcards

3. Haematology + Oncology Flashcards

1
Q

What risk factors are there for developing iron deficiency anaemia?

A

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

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2
Q

How does iron deficiency anaemia present?

A

-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

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3
Q

How is anaemia diagnosed?

A

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)

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4
Q

How should you treat anaemia?

A

-5mg/kg elemental iron/day - oral ferrous salt
-Response seen in 5-10 days (continue for 3 months after Hb has normalised
-Dietary counselling

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5
Q

How do brain tumours normally present?

A

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

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6
Q

What are low grade gliomas and what is their prognosis?

A

-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

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7
Q

What are high grade gliomas and what are their prognosis?

A

-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

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8
Q

What is a diffuse brainstem glioma?

A

-Glioma in pons - usually high grade and inoperable
-RT mainstay of treatment
-Median survival <1yr

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9
Q

What is a primitive neuroectodermal tumour and what is their prognosis?

A

-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

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10
Q

What is an ependyma and what is its prognosis?

A

-10% of CNS tumours
-Periventricular sites
-Obstructive hydrocephalus at presentation
-10% –> spine mets
-Surgical excision + RT
->70% survival if complete excision

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11
Q

What are CNS germ cell tumours?

A

-5% of CNS tumours
-Secreting tumours - raised markers
-Surgery for teratomas, CT+RT for others

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12
Q

What is a craniopharyngioma?

A

-10% of CNS tumours
-Arise from squamous remnant of Rathke’s pouch
-Locally invasive
-Visual field loss, pituitary dysfunction
-Surgery + RT

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13
Q

What is a retinoblastoma and what is its prognosis?

A

-3% of all tumours
-Present with squint / visual disturbances and abnormal light reflex
-Surgery, chemo, focal therapy
-90% 5YSR

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14
Q

What is causes haemophilia and what is its inheritance pattern?

A

-X-linked recessive inheritance
-H-A = defective production of Factor VIII
-H-B = deficiency in Factor IX (less common)
-Carrier females

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15
Q

How does the severity of Haemophilia vary?

A

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

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16
Q

What are haemarthroses?

A

-Bleeding into joints
-Joint becomes painful, swollen, tender and warm
-Severe limitation of movement
-May be unable to weight bear

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17
Q

How would you investigate haemophilia?

A

-APTT (increased), FVII
-PT (normal)
-Plasma FVIII and IX assay
-Cranial CT if any suspicion of bleed
-USS if ?haemarthroses

18
Q

How would you manage haemophilia?

A

-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

19
Q

What is Henoch Schonlein purpura?

A

-Small vessel vasculitis - associated with IgA
-Commonly follows an URTI / exposure to drug / allergen
-Most commonly affects pre-pubertal boys

20
Q

How does Henoch Schonlein purpura present?

A

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%

21
Q

How would you investigate Henoch Schonlein purpura?

A

-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

22
Q

How would you treat Henoch Schonlein purpura?

A

-Most are self-resolving within 6 weeks
-NSAIDs for arthralgia
-Steroids for any abdominal pain accompanied by N+V

23
Q

What are the differences between different forms of leukaemia?

A

-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

24
Q

How does leukaemia present?

A

-Malaise
-Anorexia
-Anaemia - lethargy and pallor
-Neutropenia - frequent / severe infections
-Bruising
-Bone pain
-Lymphadenopathy / splenomegaly / hepatomegaly
-Testicular swelling

25
Q

How is leukaemia diagnosed?

A

-FBC - anaemia, thrombocytopenia, neutropenia
-Bone marrow biopsy - morphology, immunophenotype, cytogenetics
-CSF - cytospin
-CXR - ?mediastinal mass

26
Q

How would you treat leukaemia?

A

-Chemo - complete remission in 95% of children
-Intensification chemo - maintains remission
-Methotrexate / cranial irradiation - protects CNS from involvement
-Monthly cycles of maintenance chemo
IF RELAPSE
-High dose chemo
-Allogenic bone marrow transplantation

27
Q

How does the incidence of non-Hodgkin’s lymphoma vary from Hodgkin’s?

A

NHL = more common in young children
HL = more common in adolescents / young adults and those with previous EBV infection

28
Q

How does the presentation of non-Hodgkin’s lymphoma vary from Hodgkin’s?

A

NHL:
-Peripheral lymph node enlargement
-Intrathoracic / mediastinal / abdominal / gut / lymph node masses
HL:
-Progressive, painless lymph node enlargement (most commonly cervical or mediastinal)
-Fever, night sweats, weight loss common in advanced stages
-Uses Ann Arbor staging

29
Q

How does the treatment of non-Hodgkin’s lymphoma vary from Hodgkin’s?

A

NHL:
-Chemo (similar to ALL)
HL:
-Chemo
-RT
-Bone marrow transplant if advanced / recurrent

30
Q

How does the investigation of non-Hodgkin’s lymphoma vary from Hodgkin’s?

A

NHL:
-Bone marrow aspirate
-LP
-Pleural and abdominal fluid aspirate
-CT and PET scans
HL:
-CT neck, chest, abdo, pelvis + PET scan
-Bone marrow aspirate
-EBV serology
-ESR
-Isotope bone scan if advanced / ?bone spread
-Histology of lymph nodes

31
Q

What causes sickle cell anaemia?

A

-Autosomal recessive condition
-Defect in beta chain of HbA
-Sickle shaped RBCs increase blood viscosity –> vaso-occlusion and reduced flow through small vessels –> tissue infarction
-RBCs are prematurely destroyed –> haemolytic anaemia –> trapped in microcirculation –> ischaemia
-Also hyposplenic –> high risk of infection

32
Q

What are the clinical features of sickle cell anaemia?

A

-Don’t develop symptoms until aged 4-6months when abnormal HbS takes over from foetal Hb
INFANTS
-Dactylitis
-Splenic sequestration
-Pneumococcal sepsis
YOUNG CHILDREN
-Parvovirus infection
-Painful crises in long bones precipitated by infection, dehydration, deoxygenation
-Stroke
-Delayed growth and development
OLDER CHILDREN
-Painful crises
-Avascular necrosis
-Stroke
-Renal impairment, retinopathy, ENT problems

33
Q

How is sickle cell diagnosed?

A

-Blood film
-Prenatal diagnosis may be done
-Routine screening of Afro-Caribbean children

34
Q

How do you manage an acute sickle cell crisis?

A

-Analgesia
-Antihistamine
-Supportive care, correction of cause (o2, hydration, abs)
-Blood products

35
Q

What is the maintenance therapy for sickle cell?

A

-Avoid precipitating (cold, hypoxia, dehydration)
-Hydroxycarbamide, L-glutamine
-Repeated blood transfusion
-Vaccinations, prophylactic penicillin
-Daily oral folic acid
-Bone marrow transplant –> curative

36
Q

What are the 6 main types of sickle cell crisis?

A

PAINFUL = vaso-cclusion causing ischaemia + pain in bones + abdomen
APLASTIC = temporary bone marrow failure –> severe anaemia (triggered by parvovirus B19)
SPLENIC SEQUESTRATION = life-threatening sudden enlargement of the spleen –> hypovolaemia
CEREBROVASCULAR = vaso-occlusion in cerebral circulation, cause of significant mortality
PRIAPISM = persistent and painful erection of penis, risk of impotence

37
Q

What is a neuroblastoma and how does it present?

A

-Malignant embryonal tumour
-Usually presents in children <5yrs
-Most commonly arises in the adrenal glands and sympathetic nervous system
PRESENTATION
-Abdo pain / distension / mass
-Skin nodules, periorbital bruising (panda eyes)
-May cause Horner’s syndrome / spinal cord compression
-Pallor, weight loss
PROGNOSIS
-Survival >90% in low-risk cases, only cured in 20-30% of all cases

38
Q

What causes thalassaemia and what are its effects?

A

-Inherited defect in synthesis of global chains
-Ineffective erythropoiesis –> precipitation of excess chains –> haemolysis –> variable severity anaemia

39
Q

What are the different types of thalassaemia?

A

ALPHA
-Most are asymptomatic
-Anaemia and splenomegaly
BETA (most common)
-Abdo distension, mild microcytic anaemia
-Lethargy, failure to grow / gain weight
-Jaundice, misaligned teeth
HOMOZYGOUS BETA
-Severe haemolytic anaemia –> compensatory bone marrow hyperplasia
-Marked hepatosplenomegaly

40
Q

How would you manage thalassaemia?

A

-Regular blood transfusions - give with chelating agent desferrioxamine due to risk of iron overload
-Folic acid supplement

41
Q

What is a Wilms tumour and how does it present?

A

-Embryonal tumour of the kidney (nephroblastoma)
-Most occur in children <7yrs
-Metastatic disease occurs in <10%
PRESENTATION
-Visible, palpable, unilateral, painless abdominal mass
-Haematuria and HTN

42
Q

How would you investigate and manage a Wilms tumour?

A

INVESTIGATIONS
-Abdo USS, CT, CXR
-Blood count, coag studies, renal function
MANAGEMENT
-Surgical excision + chemo
-RT used in incomplete resection

Y4 Paeds CCs (25 decks)

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