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Acute Lymphoblastic Leukaemia (Epidemiology (The commonest cancer in…
Acute Lymphoblastic Leukaemia
Epidemiology
The commonest cancer in childhood
Thought to develop from a combination of genetic susceptibility and an environmental trigger
Malignancy of immature lymphoid cells (gives rise to T cells and B cells)
CNS involvement is common
Most common between 2 and 4 years of age
Ionising radiation and Down's syndrome are important associations
Pathophysiology
Majority of cases derive from B-cell precursors
There is increased proliferation of immature lymphoblast cells (B or T cell precursors) in bone marrow
Affects B or T lymphocyte cell lines, arrests the maturation and promotes uncontrolled proliferation of immature blast cells (immature precursor of myeloid cells or lymphoid cells)
Clinical Presentation
Node infiltration resulting in lymphadenopathy
CNS infiltration resulting in headache and cranial nerve palsies
Liver/spleen infiltration resulting in hepatosplenomegaly
Mediastinum infiltration resulting in mediastinal masses with superior vena cava obstruction
Bone marrow infiltration resulting in bone pain
Marrow failure
Infection - low WCC
Resulting in infections
There is fever and mouth ulcers
Bleeding - low platelets
Resulting in bleeding and bruising
Anaemia (low Hb)
Resulting in breathlessness, fatigue, angina and claudication
There is pallor and cardiac flow murmur
Diagnosis
CXR and CT scan to look for mediastinal and abdominal lymphadenopathy
Lumbar puncture to look for CNS involvement
FBC & Blood film
WCC is usually high
Blast cells on film and in bone marrow
Treatment
ALLOPURINOL (prevent tumour lysis syndrome)
IV fluids - insert Hickman line so can easily take blood for testing and administer drugs/fluids
Neutropenia may lead to deadly infections - treat with prophylactic antivirals, antibacterial and antifungals
Chemotherapy
Blood and platelet transfusions
Marrow transplantation