Leukaemia

Question 1

Leukaemia?
Types?

Answer 1

Leukaemia is the name for cancer of a particular line of the stem cells in the bone marrow. This causes unregulated production of certain types of blood cells. Types of leukaemia can be classified depending on how rapidly they progress (chronic is slow and acute is fast) and the cell line that is affected (myeloid or lymphoid).

Question 2

Types of leukaemia?
Ages?

Answer 2

The types of leukaemia that affect children from most to least common are:

Acute lymphoblastic leukaemia (ALL) is the most common in children
Acute myeloid leukaemia (AML) is the next most common
Chronic myeloid leukaemia (CML) is rare

Rarer and very specialist leukemias exist, but you are very unlikely to encounter them.

ALL peaks aged 2 – 5 years
AML peaks aged under 2 years

Question 3

Pathophysiology leukaemia?

Answer 3

Leukaemia is a form of cancer of the cells in the bone marrow. A genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell.

The excessive production of a single type of cell can lead to suppression of the other cell lines, causing underproduction of other cell types. This results in a pancytopenia, which is a combination of low:

Red blood cells (anaemia),
White blood cells (leukopenia)
Platelets (thrombocytopenia)

Question 4

Risk factors leukaemia?

Answer 4

Radiation exposure, for example with an abdominal xray during pregnancy, is the main environmental risk factor for leukaemia.

There are several conditions that predispose to a higher risk of developing leukaemia:

Down’s syndrome
Kleinfelter syndrome
Noonan syndrome
Fanconi’s anaemia

Question 5

Presentation of leukaemia?

Answer 5

The presentation of anaemia is typically non-specific. Symptoms can include:

Persistent fatigue
Unexplained fever
Failure to thrive
Weight loss
Night sweats
Pallor (anaemia)
Petechiae and abnormal bruising (thrombocytopenia)
Unexplained bleeding (thrombocytopenia)
Abdominal pain
Generalised lymphadenopathy
Unexplained or persistent bone or joint pain
Hepatosplenomegaly

Question 6

Diagnosis of leukaemia?

Answer 6

NICE recommend referring any child with unexplained petechiae or hepatomegaly for immediate specialist assessment.

If leukaemia is suspected based on the non-specific signs above, NICE recommend a very urgent full blood count within 48 hours.

Investigations to establish the diagnosis:

Full blood count, which can show anaemia, leukopenia, thrombocytopenia and high numbers of the abnormal WBCs
Blood film, which can show blast cells
Bone marrow biopsy
Lymph node biopsy
Further tests may be required for staging:

Chest xray
CT scan
Lumbar puncture
Genetic analysis and immunophenotyping of the abnormal cells

Question 7

Management of leukaemia?

Answer 7

Treatment of leukaemia will be coordinated by a paediatric oncology multi-disciplinary team. Leukaemia is primarily treated with chemotherapy.

Other therapies:

Radiotherapy
Bone marrow transplant
Surgery

Question 8

Complications of chemotherapy?

Answer 8

Failure to treat the leukaemia
Stunted growth and development
Immunodeficiency and infections
Neurotoxicity
Infertility
Secondary malignancy
Cardiotoxicity

Question 9

Prognosis of leukaemia?

Answer 9

The overall cure rate for ALL is around 80%, but prognosis depends on individual factors. The outcomes are less positive for AML.