Hamish, 5, has been tired and miserable for the last week of the school holidays. On the second day back at school, his Mum is asked to collect him after a bleeding nose that lasts about fifteen minutes. The teacher comments to Mum that Hamish is looking a bit “thin & pale”, and they’ve noticed a lot of bruising on his shins.
The GP agrees and orders a full blood count, which shows: Hb 50 g/L (100 – 150); Plt 2 x109/L (150 – 450); WCC 45.8 x109/L (80% lymphocytes, 30% blasts); “Blasts seen on film”.
ALL is the most common childhood haematological malignancy
Paediatric oncology is strongly consultant-driven
First presentation to the tertiary oncology centre is extremely stressful and a medically intense time
Aim is to achieve remission in induction
ALL may present in a broad variety of signs & symptoms<
The GP phones you, the Paeds Oncology registrar, with these results, and Hamish soon arrives into Emergency. You phone the consultant, who attends to meet family, take the history and examine Hamish.
Hamish looks pale but bright eyed. Vitals are 36.9oC, HR 130, RR 25, SaO2 97%, BP is normotensive with brisk capillary refill.
Has been grizzly and “not himself” for the last ten days: picking at food; complaining of sore legs for 2/7. Not recently unwell/coryzal symptoms/diarrhoea. No wheeze. No steroid exposure. No blurry vision. Developmentally meeting milestones. No FHx of childhood malignancy.
(When taking the history, specifically ask about B symptoms: fever, night sweats, and weight loss).
Pale boy with signs of weight loss. Bruising of the elbows, knees and legs. HS 2+ flow murmur. Lungs – no wheeze, good equal air entry. Abdomen soft, bowel sounds, liver 5cm below the costal margin, spleen 8cm below costal margin, not tender. Enlarged inguinal nodes bilaterally. Testicular examination – normal size for age. Aside from the bruising, you identify no areas of broken skin, boils, erythema or rashes. ENT examination is unremarkable. Fundoscopy unremarkable.
(Also, note any dysmorphism, Tanner stage, Lansky performance score).
What is ALL?
Acute lymphoblastic leukaemia is the most common childhood cancer. It is bimodal in incidence in childhood with peaks at around 2 years, and then at around 16 years of age.
ALL accounts for around 80% of childhood leukaemias, the remainder being acute myeloid leukaemia and rarer types. Approximately 85% of children with acute lymphoblastic leukaemia have B-cell ALL, with ~15% having T-Cell ALL. 2-3% will have Burkitt lymphoma, a mature B-cell leukaemia, treated differently from most leukaemias.
How does ALL usually present?
The most common presentations are with bone pain. Many children experience bone aches due to ‘growing pains’, so it’s important to know how to differentiate bone pain related to oncology issues, and growing pains.
- bone pain tends to wake you up in the middle of the night, whereas growing pains are usually felt more when the child is falling asleep
- children with growing pains should not have difficulty walking
- growing pains tends to present as a pattern i.e. same type of pain at the same of day
- children with growing pains will have completely normal blood counts
- there should be no fever or weight loss associated with growing pains
As with this case, ALL can also present with bleeding. Other presentations include splenomegaly (10-20%), mediastinal mass, renal failure (due to hyperuricaemia), or leukostatic symptoms (respiratory distress, altered mental status) in patients with a high WCC .
Rarely patients who initially are thought to have ITP actually turn out to have ALL.
ALL can also include extramedullary sites e.g. CNS, testes, liver/spleen, kidneys, skin (rare). With this in mind, the list of presenting features include…
- Typically weight loss (or failure to thrive), anaemia, fatigue will be present
- Bone or joint pain
- Epistaxis or bleeding gums
- Recurrent fever (low grade)
- Persistent cough
- Lymphadenopathy (including tonsillar hypertrophy)
- Wheeze (from a mediastinal mass) or
- Blurry vision/diplopia
- Testicular enlargement
- Headaches (with papilloedema & retinal haemorrhages)
- Respiratory distress (hyperviscosity)
- Cranial nerve palsies
Practical points at diagnosis
For some paeds oncology departments, there is a policy that the most senior ED doctor should place the cannula for a patient’s first presentation. In a stressful time for the child and family, this is a drip that needs to go in first time with as little fuss as possible. It is important for the medical staff to build trust with child and family early.
Although most specialties would have the registrar or senior resident “do the admission”, oncologists will often meet the family as soon as they are referred. The family will be seeing a lot of their oncologist, and establishing trust and rapport very early in the piece is important.
In this kind of presentation – from the community in a stable child during daylight hours – the oncologist will often have spoken to the haematologist about the film prior to meeting the patient. This enables them to give the most likely diagnosis (based on the film and history/examination) & answer a few questions.
National Cancer Institute – Childhood Acute Lymphoblastic Leukaemia Treatment (PDQ) – Risk-based Treatment Assignment
Peppercorn J et al. Comparison of outcomes in cancer patients treated within and outside clinical trials: conceptual framework and structured review. Lancet 2004; 363: 263–70
QPHON Guide to the Care of Children with Cancer in Queensland Document No. 2.1 15062012 © 2012 State of Queensland Queensland Health. via Q-Health intranet.