Oncology & Haematology Flashcards
List the possible features of Acute Lymphoblastic Leukaemia (regarding infiltration sites) (10)
General: Malaise + Anorexia
Marrow infiltration: Thrombocytopenia (bruising, petechiae) Neutropenia (infection) Anaemia (lethargy) Bone pain
Reticulo-endothelial infiltration:
Hepatosplenomegaly
Lymphadenopathy
Other organ infiltration:
Testes enlargement
CNS (palsies, headache, vomiting)
What is the peak age of ALL presentation?
Can occur all ages but peak age 2-5y/o
What Ix can be done in ALL? (3)
FBC - abnormal
Bone marrow biopsy (confirm Dx + grade)
CXR (id mediastinal masses = T-cell disease)
What are the diff tumour cell types in ALL? (3)
How does the type affect Tx approach?
L1 - small uniform cells
L2 - large varied cells
L3 - large varied cells with vacuoles
T-cell → cyclophosphamide + intense asparaginase Tx
Mature B-cell → treat like lymphoma (short-term intensive + high dose MTX)
What are the 5 general regimes / steps in treating ALL?
Ray Is the Crazy Cancer Teacher
Remission induction
Intensification (to consolidate remission)
CNS (intrathecal chemo)
Continuing therapy (PCP prophylaxis)
Treatment of relapse (high dose chemo, total body irradiation + marrow transplant)
What are the main prognostic factors in ALL (7)
Gender: male
Age: <1 or >10
Tumour load (measured by WBC)
Spread: CNS involvement worse
Cytogenetic abnormalities in tumour cells
Speed of response to initial chemo
Minimal residual disease assessment (high submicroscopic levels leukaemia on PCR)
What are the haematological differences b/wn fetal/neonatal and adult blood? (4)
Fetus haemopoiesis in liver → bone marrow
HbF (2a2y - higher O2 aft) → HbA (2a2B) by 1yr old
Hb: high (14-21 compensates for low O2 conc) → low (10 RBC production)
WCC in neonates higher than in older children
Platelet counts sim to adults
What are the main complications of ALL treatment? (4)
Neutropenic sepsis
Hyperuricaemia (tumour lysis syndrome - give allopurinol)
Poor growth
CNS infiltration / relapse
In anaemia, what are some causes of:
Microcytic (2)
Macrocytic (3)
Normocytic (2)
MCV < 70 : Fe defc anaemia, Thalassaemia
MCV > 100 : Folate defc, B12 defc, Haemolysis
MCV 80-100 (normal): Haemolysis, Marrow failure
What are the causes of Fe defc anaemia in children?
Inadequate intake
Malabsorption
(Common in infants as require more + more to accompany blood volume growth)
(Can develop due to delayed weaning >6m)
What dietary advice can be given to prevent Fe defc anaemia? (5)
Avoid cow's milk if <1yr If formula-fed - iron fortified If breast fed - wean at 4-6m Adequate Vit C intake Iron supplements (if premature)
At what Hb do symptoms of Fe defc anaemia appear?
What are the features of Fe defc anaemia? (4)
Tires easily
Feeds slower than usual
Pallor (conjunctiva / tongue/ palmar)
Pica (eating dirt)
What should be Ix if normal dietary changes / supplements don’t improve a child’s Fe defc anaemia? (2)
Malabsorption (coeliac)
Bleeding (oesophagitis, Meckel’s diverticulum)
What is usually the cause of haemolysis in neonates?(1) + in children? (3)
Neonates - autoimmune haemolytic anaemia
Children - RBC membrane/enzyme disorders (hereditary spherocytosis, G6PD defc, PK defc), haemoglobinopathies
What are the features of haemolytic anaemia (5)
Jaundice (raised unconj bili + urobili) Gallstones (raised bill) Anaemia Hepatosplenomegaly (reticulo-endothelial hyperplasia) May present with aplastic crisis
What Ix can be done for haemolytic anaemia?
What Tx?
Ix - blood film, Coombs test (+ve in autoimmune)
Tx - folate supplements (mild), splenectomy (severe)
What is the incidence of hereditary spherocytosis?
What is the pathophysiology?
What can trigger severe anaemia due to HS?
1 in 5000 (3 dominant: 1 spontaneous)
Defective RBC skeletal prots → RBC loses some memb + becomes spheroidal as passes thru spleen + destroyed
Parovirus 19 can → severe anaemia (aplastic crisis)
How does G6PD defc present? (4)
Neonatal jaundice - 1st 3/7 OR precipitated by infection/drugs/broad beans
Fever
Malaise
Haemoglobinuria
What is G6PD defc?
Common RBC disorder
X-linked
High prevalence in central Africa
G6PD stops oxidative damage to cell
What is Pyruvate Kinase (PK) defc?
2nd commonest cause haemolytic anaemia
Due to lack of glycolysis enzyme → decrease in ATP → cell becomes rigid → spleen destruction
What mutation has occurred in sickle cell anaemia?
What ethnicities is it commoner in?
Point mutation = glutamine → valine
Common in Afro-Caribbean / black
What are the 4 types of sickle cell disease / trait? (+their Hb status)
Sickle cell anaemia - HbSS (homozygous) mutation in both B-globin genes → some HbF + no HbA
HbSC (HbB = diff point mutation in B-globin → no normal B-globin genes → no HbA)
Sickle B-thalassaemia → no normal B-globin (sim symps to HbSS)
Sickle trait → 60% blood HbA (40%HbS) → asymp (only ID by blood test)
How does HbS end up causing symptoms?
HbS → sickle shaped RBCs
→ Have reduced life span
→ Can trap in microcirculation → vessel occlusion + ischaemia in organ/bone
What are the features of sickle cell disease (6)
Painful (vaso-occlusive) crises
Splenomegaly - in younger
Infection susceptibility to pnuemococci/HiB (hyposplenism from micro infarcts)
Priapism (erection) - treat asap or later erec dysfunc
Anaemia: moderate or acute (sudden drop)
Clinical jaundice (from haemolysis)
What are some of the causes of acute anaemia in sickle cell disease? (3)
Haemolytic (infection)
Aplastic (parovirus)
Sequestration (sudden hepatosplenomegaly, abdo pain + circ collapse)
List the long-term complications of sickle cell disease (9)
Short stature
Gallstones
Heart failure (from uncorrected anaemia) Cardiomegaly (chronic anaemia)
Adenotonsilalr hypertrophy Renal dysfunc Leg ulcers Stroke/cognitive probs Educational/behav probs
What are some of the triggers of a sickle cell crisis? (5)
Cold Stress XS exercise Infection Hypoxia
How is a sickle cell crisis managed?
What are the other aspects of management in sickle cell anaemia? (3)
Crises: analgesia + maintain good hydration + O2
Prophylaxis: full imms + penicillin throughout childhood Folic acid (due to increased demand) Avoiding crises triggers
What ethnicity is a-thalassaemia major mostly seen in?
What are the features (of major)?
SE Asian
Fetal hydrops in 2ndT (fetal anaemia → oedema + ascites)
3 deletions → mild/mod anaemia
2 deletions → asymp
What are the 2 main types of B-thalassaemia? What are their Hb status?
B-thalassaemia major - abnorm B-globin gene/production
→ HbA not produced
B-thalassaemia intermedia - B-globin mutations
→ little HbA + lots HbF (milder/variable severity)
What biochemical signs would be seen in someone with B-thalassaemia trait? (3)
What condition may it be confused with?
Hypochromic
Microcytic
Anaemia absent (→disproportionate with MCH/MCV)
Can confuse with Fe defc (here Ferritin stores normal)
What are the clinical features of thalassaemia? (5)
Severe anaemia (transfusion dependant from 3-6m)
Jaundice
Failure to thrive
Extramedullary haemopoiesis:
Bone marrow expansion (maxillary overgrowth + skull bossing)
Hepatosplenomegaly
What are the chances of a child being affected/carrier in heterozygous parents of thalassaemia?
Affected: 25% chance
Carrier: 50% chance
→ therefore offer prenatal Dx
What are the management options in thalassaemia? (2)
Lifelong monthly transfusions (or else fatal) Marrow transplant (curative - from compatible sibling)
What are some complications of repeat transfusions in thalassaemia?
+ how these treated?
→ Chronic Fe overload:
Cardiac failure, Cirrhosis, DM, Growth failure, infertility
Treat with Iron chelation (removes XS Fe)
What are the main features of bleeding disorders? (5)
Bruising Petechiae Purpura Mucosal bleeding (epistaxis, gums etc) Major haemorrhage (GI bleed, intracranial - rare)
What is the bleeding risk in platelets:
<20?
20-50?
50-150?
<20 → high spontaneous bleeding risk
20-50 → high risk excess bleeding in trauma/surgery
50-150 → low risk bleeding (unless major trauma/surg)
What happens in immune thrombocytopenia purpura?
What is the incidence?
ITP: anti-platelet IgG autoantibodies destroy circulating platelets (→ increased megakaryocytes)
Incidence 1 in 25,000
How do children with ITP usually present? (age + features)
B/wn 2-10yrs
Post-viral infection
Features of bleeding disorders:
Bruising/petechiae/purpura / mucosal bleeding / GI bleed
What are some atypical features of ITP? (4)
Anaemia
Neutropenia
Hepatosplenomegaly
Marked lymphadenopathy
What are some DDx of ITP?
ALL (req. marrow biopsy if lymphadenopathy)
Aplastic anaemia
SLE
How is ITP managed?
80% self-limiting within 6-8wks
Only req admission if major haemorrhage/persistent minor
What is DIC
= disorder of coag pathway activation → diffuse fibrin deposition in microvasc + consumption of coag factors/platelets
What things could cause DIC in children (2)
Severe sepsis e.g. meningococcal septicaemia
Shock from circulatory collapse e.g. extensive tissue damage from trauma/burns
What are the clinical features of DIC (3)
What biochemical markers should you suspect DIC? (7)
Bruising / Petechiae / Purpura
D-dimers Reduced platelets Reduced fibrinogen (/raised products) Reduced antithrombin Reduced Protein C + S Prolonged PT/APTT Microangiopathic haemolytic anaemia
How is DIC managed? (2)
- treat underlying causes e.g. sepsis (in ICU)
2. Supportive care: FFP, platelets, coag factor transfusion
What is the factor defc in Haemophilia A + B? + their incidences
Haemophilia A: factor 8, 1 in 5000
B: factor 9, 1 in 30,000
How does haemophilia present?
In neonatal: intracranial haemorrhage / oozing from heel prick In older (when walking starts): crippling arthritis (bleeding in joints/muscles)
How is Haemophilia managed? (4)
Prophylactic factor 8
Recombinant factor 8/9 (IV, when bleeding)
Desmopressin (allows mild haemophilia w/o recombinant)
Physio - preserve muscle strength / avoid immob damage
What are the indications for splenectomy? (4)
Hereditary spherocytosis
IPT (Chronic)
Lymphoma
Spleen trauma + uncontrolled bleeding
What prophylactic measures must be done before/after a splenectomy?
Before: imms to pneumococcus, HiB, meningococcus, influenzae
After: lifelong Abx + malaria precautions abroad
Which forms of lymphoma are commoner in young/older children
What is the cure rate in both types
Younger = NHL
Older (adolescent) = Hodgkin’s
Both >80% survival rate
How does Non-Hodgkins lymphoma present?
Depends on T/B cell
T cell (same spectrum as ALL): Mediastinal mass (poss → SVC obstrn) Marrow infiltration
B cell:
Localised LN disease (head/neck/abdo (pain/intuss))
What cells are involved in Hodgkin’s lymphoma?
How does it present?
Reed-Sternberg cells
Painless lymphadenopathy (hard/firmer than benign) Starts in upper body (neck) + spreads down slowly RARELY systemic symptoms (anorexia, wt loss) - even advanced
List the short term SEs of chemo (8)
Hair loss
Sore mouth
N + D + V
Appetite/wt loss
Anaemia
Infection (neutropenia)
Bruising
Mood instability/irritability
List the long-term SEs of chemo (6)
Cancer recurrence
Psychological effects
Subfertility
Reduced growth
Delayed puberty
Toxicity: neuro / hepato / renal / cardio / pulm
What tissue does neuroblastoma originate from?
Neural crest tissue in adrenal medulla + sympathetic nervous tissue
How does a primary tumour present in neuroblastoma? (4)
Abdo mass (can be along symp chain neck→pelvic, classically adrenals)
Mass often large/complex, crosses midline + major vessels/LNs
If invade adj intervert foramen → spinal cord compression
Limp
Hepatomegaly
How does neuroblastoma present if metastatic? (4)
> 2yrs old
Bone pain
Marrow suppression
Wt loss / malaise
(+ Limp
+ Hepatomegaly (neurblastoma progressed))
What syndromes is Wilm’s tumour (nephroblastoma) associated with?
Overgrowth syndromes
T18
What are the presentational features of Wilm’s tumour? (7)
<5yrs (>80% cases)
Abdo mass (incidental find) Abdo pain Anorexia
Haematuria
Hypertension
Anaemia
What will be shown on scan in Wilm’s tumour
What is the prognosis like
USS and/or CT/MRI
→ Shows intrinsic renal mass distorting normal structures
> 80% cured (60% if metastasised)
However often recur so worse prognosis
Where does a rhabdomyosarcoma originate from?
What are some common sites?
Primitive mesenchymal tissue (various primary sites - each with diff presentation/prognosis)
Head+neck (40%)
GU (bladder/ female GU tract/ paratesticular)
How does a rhabdomyosarcoma present in head+neck? (3)
Proptosis
Nasal obstrn
Blood stain nasal discharge
How does rhabdomyosarcoma present in GU? (4)
Dysuria
Urinary obstrn
Blood stained vag discharge
Scrotal mass
How does an osteosarcoma present?
Persistent localised bone pain
Usually limbs
Pain preceding detection of mass
Pt otherwise well
What Ix should be done in osteosarcoma?
Bone X-Ray (shows destruction + new periosteal home formation) Chest CT (lung metastases)
What is the cause of retinoblastoma?
Hereditary (100% in bilateral, 20% in unilateral)
Susceptibility gene on csome13 (dominant)
How does retinoblastoma present? (3)
<3yrs old
White pupillary reflex (not red)
OR a squint
What is the incidence of childhood malignancy in <15s
What are the commonest cancers in children? (3)
1 in 500 <15s
Leukaemias (32%)
Brain/Spinal (24%)
Lymphomas (10%)
What age do you see: Neuroblastoma Wilms Hodgkins Bone tumours
Neuro + Wilms <6yrs
Hodgkins + bone tumours → peak in adolescence
What type of anaemia will be seen on blood film in B12/Folate defc?
Macrocytic megaloblastic anaemia
