Acute Lymphoblastic Leukaemia

Question 1

Definition of acute lymphoblastic leukaemia

Answer 1

A malignancy of the lymphoid progenitor cells, which can affect the B or T cell lineages.
• Characterised by maturation arrest which promotes uncontrolled proliferation of immature lymphoblast cells, with BONE MARROW FAILURE and tissue infiltration

Question 2

Aetiology/pathophysiology of acute lymphoblastic leukaemia

Answer 2

• Most common malignancy of childhood

• Genetic alteration of a lymphoid progenitor cell would lead to maturation arrest and uncontrolled proliferation of the lymphoblastic cells (immature)
• These lymphoblasts would infiltrate the bone marrow and other organs (; hence disrupting their normal function)

• Split into B-cell ALL (most COMMON in adults) and T-cell ALL

Question 3

Risk factors for acute lymphoblastic leukaemia

Answer 3

• Genetics (Down’s syndrome, Klinefelter syndrome)
• Environmental (radiation exposure and smoking)
• Viral infections
• History of malignancy
• Chemotherapy treatment

Question 4

History and Examination of acute lymphoblastic leukaemia

Answer 4

• Symptoms of Bone Marrow failure:
• Anaemia:
◦ Fatigue
◦ Dizziness
◦ Palpitations
◦ Dyspnoea/SOB
◦ Pallor
• Thrombocytopenia:
◦ Easy bruising (ecchymosis)
◦ Epistaxis
◦ Petiechiae
◦ Menorrhagia
• Neutropenia:
◦ Frequent/severe infections
◦ Fever

• Organ infiltration:
◦ Lymphadenopathy: generalised, painless and moveable, common presenting complaint
◦ Hepatosplenomegaly
◦ Bone pain: due to bone marrow infiltration
◦ Renal enlargement
◦ Neurological signs: Meningitis, Papilloedema, cranial nerve palsies
◦ Testicular swelling

Question 5

Investigations for acute lymphoblastic leukaemia

Answer 5

• FBC: Likely to see normocytic normochromic anaemia, leukocytosis (high WCC), neutropenia (despite raised WBC) and thrombocytopenia
• Blood film: Would see abundant leukaemic lymphoblasts
• Bone marrow aspiration and trephine biopsy: Bone marrow hypercellularity and infiltration by leukaemic lymphoblasts is characteristic of ALL
• LFTs, U&Es: may be deranged if organ infiltration
• CXR and CT: can see mediastinal and abdominal lymphadenopathy

Question 6

Treatment for acute lymphoblastic leukaemia

Answer 6

1) Induction Therapy: Is the first phase of the treatment, consists of a CHEMOTHERAPY regime. This is to induce remission

+ CNS prophylaxis: Give intrathecal Methotrexate

+ Support: e.g blood/platelet transfusion, IV fluids, antibiotics for any infections

Question 7

Complications of acute lymphoblastic leukaemia

Answer 7

• Pancytopenia: Caused by bone marrow infiltration. Platelets can be transfused
• Infection: will be more susceptible to infection, especially due to chemotherapy, prophylaxis can be given

Question 8

Prognosis of acute lymphoblastic leukaemia

Answer 8

• 70% chance of 5 year survival with ALL.
• Cure rates are lower in adults than they are in children
• Low prognosis with older age, high WBC, CNS signs etc