Acute Lymphoblastic Leukaemia Flashcards
what is the definition of ALL?
Cancer of lymphoblasts predominantly BM, immature lymphoid cells
what is the epidemiology of ALL?
‘Children’s leukaemia’ - Most common malignancy in children
Bimodal incidence (later in life high too)
Rare
Slight male predominance
what is the aetiology of ALL?
Radiation Benzene Smoking Downs Immunodeficiency Genetic component
what are the risk factors for ALL?
Radiation exposure. Being exposed to high levels of radiation is a risk factor for both ALL and acute myeloid leukaemia (AML) Certain chemical exposures Certain viral infections Certain genetic syndromes Age Race/ethnicity Gender Having an identical twin with ALL
what is the pathophysiology of ALL?
Affects B or T lymphocyte cell lines, arrests the maturation and promotes uncontrolled proliferation of immature blast cells (immature precursor of myeloid cells (myeloblasts) or lymphoid cells (lymphoblast))
- Majority of cases derive from B-cell precursors
- There is increased proliferation of immature lymphoblast cells (B or T cell precursors) in the bone marrow:
• If all B cells = CHILDREN
• If all T cells = Adults
what are the key presentations of ALL?
Non specific
Marrow failure - anaemia, infection, bleeding
what are the signs of ALL?
T-ALL = high blood count Pallor and cardiac murmur Liver and spleen enlargement Lymph node enlargement Mediastinum infiltration resulting in mediastinal masses with superior vena cava obstruction
what are the symptoms of ALL?
Bone pain, tired, infection, bleeding, testicular swelling
Breathlessness, fatigue, angina, claudication
Bleeding and bruising
Fever and mouth ulcers
what are the first line investigations for ALL?
FBC & Blood film:
• WCC is usually high
• Blast cells on film and in bone marrow
what are the gold standard investigations for ALL?
- CXR and CT scan to look for mediastinal and abdominal lymphadenopathy
- Lumbar puncture to look for CNS involvement
what is the differential diagnoses for ALL?
Acute myelocytic leukemia. Acute anemia. Juvenile Rheumatoid Arthritis. Leukocytosis. Mononucleosis and Epstein-Barr virus. Neuroblastoma. Non-Hodgkin Lymphoma. Osteomyelitis.
how is ALL managed?
Complicated and prolonged
Large no. weeks
Chemotherapy doses, different cycle
Number of courses, 3 courses then decide to carry on or transplant
Focus on treating the brain - relapses in CNS unless therapy directed, lumbar puncture chemo needed , treat with prophylactic antivirals, antibacterial and antifungals
BM Transplant: Matched sibling = gold standard
ALLOPURINOL (prevents tumour lysis syndrome)
Blood and platelet transfusions
how is ALL monitored?
Relapse:
More chemo or targeted drugs (MCAs)
what are the complications of ALL?
From treatment:
Risk of dying (4.7% death from treatment or leukaemia)
Interactions with antifungal treatments
Infections (immunosuppressed due to chemo)
what is the prognosis of ALL?
Children more likely to survive
Mortality reduced over last 5 years
50% cured, 50% die from treatment or cancer
