Malignant disorder of the bone marrow - commonest cancer seen within paediatric population - most common form is acute lymphoblastic leukaemia (ALL)

Malignancy of the lymphatic system - Hodgkin's - non-Hodgkin's

HaemOnc Flashcards by Rosie Middleton

Define leukaemia

Malignant disorder of the bone marrow

commonest cancer seen within paediatric population
most common form is acute lymphoblastic leukaemia (ALL)

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Epidemiology of leukaemia

Incidence of ALL peaks between 2 and 5 years of age
Males more commonly affected
Genetic diagnoses (trisomy 21) at an increased risk

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Pathophysiology of ALL

Multifactorial condition - infection, genetic predisposition and environmental exposure
Disruption in the regulation and proliferation of lymphoid precursor cells in bone marrow
- excessive production of immature blast cells
- drop in numbers of functional RBC, WBC and platelets

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Clinical features of ALL

Anaemia - lethargy, pale
Thrombocytopenia - early bruising/bleeding
Leukopenia - fevers, infections
Bone pain - due to increased pressure from hyperplastic marrow
Non-specific symptoms of malignancy - weight loss, malaise

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Ddx of ALL

Bruising
- immune thrombocytopenia - low platelets but normal RBC/WBC
- trauma - hx of injury
- non-accidental injury - normal bloods with no hx of injury
Recurrent infections
- immune deficiency - long standing hx of unusual/severe infections, failure to thrive, chronic diarrhoea
Lymphadenopathy
- reactive lymphadenopathy - hx of recent infections
CNS symptoms
- infection
- raised intra-cranial pressure
Pancytopenia
- other malignant processes into bone marrow
- aplastic anaemia

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Investigations of ALL

FBC 
- pancytopenia
- anaemia
- thrombocytopenia
- significant lymphocytosis
Blood film
- presence of blast cells
CXR
- exclude mediastinal mass
Bone marrow aspirate/trephine
- confirms diagnosis
Lumbar puncture
- check for CNS involvement

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Risk scoring for ALL

Age
- children between 1-10 years old have better prognosis
WCC > 50
- at presentation gives poorer prognosis
Gender
- females have better prognosis
CNS involvement
- presence of blasts within CSF a/w poorer prognosis
Leukaemia characteristics
- morphology and cytology influences prognosis

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Mx of ALL

Immediate
- hyperhydration - to prevent hyperviscosity if have very high WCC
- steroids - if risk of airway compromise due to mediastinal mass
- abx - if infection
Definitive
- UKALL 2011 protocol
- chemotherapy - IV, oral and intra-thecally (into CSF)
- blood products - RBC, platelets
- prophylactic anti-fungal therapy

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Prognosis of ALL

90% of children survive

Should have regular follow-up and assessment for 5 years then yearly review

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Complications of ALL

Infertility
Avascular necrosis
Peripheral neuropathy
Anxiety

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Define lymphoma

Malignancy of the lymphatic system

Hodgkin’s
non-Hodgkin’s

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Epidemiology of lymphoma

Over 10% of childhood cancers

more commonly seen in boys
more common in older children
just over half childhood lymphoma are non-Hodgkin

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Pathophysiology of lymphoma

Multifactorial - infection, genetic and environmental exposures

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Risk factors for lymphoma

Epstein-Barr virus
Immunosuppression
Other cancers

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Clinical features of lymphoma

Visible or palpable mass
- non-tender lymphadenopathy
- mediastinal lymphadenopathy - cough, wheeze, SVCO
History of B symptoms
- weight loss
- nights sweats
- fevers

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Differential diagnosis for lymphoma

Reactive lymphadenopathy
- hx of recent infection
Lymphadenitis
- tender and fluctuant lymph nodes
Leukaemia
- lymphadenopathy associated with signs and symptoms of anaemia/thrombocytopenia

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Ix for lymphoma

FBC - infection
U+Es - tumour lysis syndrome due to rapid cell turnover
LDH elevated
USS - identify other nodes and assists with biopsy
CXR - mediastinal involvement
Lymph node biopsy - definitive diagnosis

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Risk scoring for lymphoma

Stage 1 - disease present in a single lymph node or organ
Stage 2 - disease present in 2 or more groups of lymph nodes or organs on the same side of the diaphragm
Stage 3 - disease present in lymph nodes or organs on both sides of the diaphragm
Stage 4 - diffuse involvement of lymph nodes and organs - liver and bone

Mx of lymphoma

Immediate
- mediastinal mass - high dose steroids and airway support
- SVCO - stenting of veins
- tumour lysis syndrome - hyperhydration, allopurinol or rasburicase
Definitive
- chemotherapy and possibly radiotherapy

Prognosis of lymphoma

Majority of children with recover

- Hodgkin’s lymphoma more favourable prognosis than non-Hodgkin’s

Complications of lymphoma

Tumour lysis syndrome
- rapid lysis of tumour cells releases large amounts of phosphorus, potassium and calcium
- can lead to kidney damage
- most likely to occur when chemotherapy first commenced
Neutropenia
Alopecia
Sub-fertility

Define nephroblastoma

Wilm’s tumour
Most common renal tumour affecting children
Usually occurs unilaterally
- can be bilateral

Epidemiology of nephroblastoma

80 children per year in the UK
Presents at pre-school age
Slight female predominance

Pathophysiology of nephroblastoma

Many tumours arise from nephrogenic rests
- embryonal remnant seen in around 1% of infants at birth
Genetics
- mutations of common tumour suppressor genes

Risk factors for nephroblastoma

Genetic and overgrowth syndromes - WAGR - Wilm's tumour, Aniridia, Genitourinary malformation and Retardation - Denys-Drash - Beckwith-Wiedemann

Clinical features of nephroblastoma

``` Abdo mass - incidental finding or by parents on otherwise well child Abdo swelling, abdo pain Fever Haematuria ```

Ddx for nephroblastoma

``` Polycystic kidney disease Hydronephrosis Neuroblastoma - abdo mass in pre-school aged child - hypertension, abdo pain and fever - periorbital ecchymosis ```

Ix for nephroblastoma

Urine dip - haematuria Uss CT/MRI - more detailed and for staging Biopsy for definitive diagnosis

Staging of nephroblastoma

1 - tumour confined to kidney and can be completely removed with surgery 2 - tumour has spread beyond kidney but can be completely removed with surgery 3 - tumour cannot be completely removed with surgery as has spread to neighbouring lymph nodes or ruptured before/during surgery 4 - distant metastases - most commonly lungs 5 - bilateral tumours

Mx of nephroblastoma

Supportive care - ensure nutrition and hydration optimised Stage 1 and 2 = soley surgery - preserve renal function whilst removing malignant lesion - commonly nephrectomy Chemo - reduced volume of malignant tissue before surgery or treat any areas of malignant disease not removed by surgery

Prognosis of nephroblastoma

Good even for metastatic disease | - 85% cured

Complications of nephroblastoma

Single functioning kidney | Cardiotoxicity due to chemotherapy

Risk factors for iron deficiency anaemia

Poor dietary intake Chronic inflammatory conditions Menorrhagia Lower socio-economic background

Clinical features for iron deficiency anaemia

``` Often incidental finding - asymptomatic Fatigue Malaise Poor exercise tolerance Irritability Headache Syncope Pallor Angular stomatitis Glossitis Nail changes Poor weight gain Tachycardia Should be no hepato/splenomegally ```

Ix for iron deficiency anaemia

``` Reduced Hb Reduced MCV Reduced ferritin Increased total iron-binding capacity Reduced transferrin ```

Mx of iron deficiency anaemia

``` Oral iron replacement - until Hb normal then 3 more months Dietary advice - green veg and red meat - better absorbed with vit C Blood transfusion rarely indicated Failure to respond to oral iron - further investigation - malabsorption - blood loss ```

Causes of haemolytic anaemia

G6PD deficiency - cells susceptible to damage from oxidation - Heinz bodies seen on blood film Hereditary spherocytosis - membrane defects make RBCs spherical - spherocytes seen on blood film Autoimmune haemolytic anaemia - antibodies to RBC membrane leads to destruction - spherocytes and reticulocytes seen on blood film

Clinical features of haemolytic anaemia

``` Fatigue Malaise Dark urine Acute haemolytic crisis - tachycardia - pallor - jaundice - fever Jaundiced sclera Splenomegaly +- hepatomegaly Gallstones - from bilirubin load ```

Mx of haemolytic anaemia

Education - triggers and signs of acute crisis Folic acid supplementation Splenectomy - if spherocytosis causing growth failure Most post-infectious autoimmune haemolytic anaemia is self-limiting

Haemolysis triggers

``` Drugs - nitrofurantoin - quinolones - sulphonamides - antimalarials Infections - EBV - mycoplasma Parvovirus B19 Food - fava (broad) beans ```

Causes of microcytic anaemia

``` Thalassaemia Anaemia of chronic disease Iron deficiency anaemia Lead poisoning Sideroblastic anaemia ```

Causes of normocytic anaemia

``` Acute blood loss Anaemia of chronic disease Aplastic anaemia Haemolytic anaemia Hypothyroidsim ```

Causes of macrocytic anaemia

``` Megoblastic - B12 deficiency - folate deficiency Normoblastic - alcohol - reticulocytosis - hypothyroidism - liver disease ```