Acute Lymphoblastic Leukaemia Flashcards
What is Acute Lymphoblastic Leukaemia (ALL)?
A malignant disease of primitive lymphoid cells (lymphoblasts) where cells do not mature, staying in blast form due to arrested maturation
What is the most common cancer in childhood?
Acute Lymphoblastic Leukaemia (ALL), peaking at 2-4 years
What genetic abnormality is associated with ALL?
The Philadelphia chromosome (t(9:22) translocation) is found in 30% of adults and 3-5% of children with ALL
Which genetic syndrome increases the risk of ALL?
Down syndrome
What type of lymphocyte is usually involved in ALL?
B-lymphocytes
How does ALL cause pancytopenia?
Excessive proliferation of malignant lymphoblasts replaces normal bone marrow cells
Name three effects of marrow failure in ALL
Anaemia, infections, and bleeding
What extra-medullary sites can ALL affect?
The CNS and testis
What are some complications of high white cell counts in ALL?
Obstruction of circulation and superior vena cava (SVC) obstruction
What findings on a blood film are characteristic of ALL?
(1) Abnormal cells (‘blasts’) -immature lymphoid cells
(2) Large size
(3) High nuclear-to-cytoplasmic ratio
(4) Prominent nucleolus
What is the diagnostic threshold for blasts in acute leukaemia?
≥20% blasts in the peripheral blood or bone marrow
Which tests are performed on a bone marrow aspirate in ALL?
(1) Morphology
(2) Immunophenotyping
(3) Cytogenetics
What is the primary treatment approach for ALL?
Multi-agent chemotherapy lasting 2-3 years, including CNS-directed treatment and immunotherapy for specific subsets
What is a Hickman line used for in ALL management?
Long-term central venous access during treatment
What are the major complications of the disease itself in ALL?
(1) Anaemia
(2) Neutropenia (leading to severe infections)
(3) Thrombocytopenia (causing bleeding)
What is the empirical treatment for neutropenic fever in ALL?
Broad-spectrum antibiotics, particularly covering gram-negative bacteria
What is Tumour Lysis Syndrome, and when does it occur in ALL?
A life-threatening condition during the first course of treatment caused by rapid cell breakdown
What are the potential late complications of ALL treatment?
Loss of fertility and cardiomyopathy
Name a common fungal infection risk in prolonged neutropenia
Pneumocystis jirovecii pneumonia (PJP)
What preventive treatment is given for PJP in ALL?
Prophylactic therapy against Pneumocystis jirovecii
Complications of treatment for Acute Lymphoblastic Leukaemia is what?
(1) Hair loss
(2) Liver and renal dysfunction
(3) Tumour lysis syndrome (during the first course of treatment)
(4) Infection
What are the clinical features of acute lymphocytic leukaemia (ALL)?
(1) Lymphadenopathy
= Swollen or enlarged lymph nodes
(2) Hepatosplenomegaly
= Enlargement of both the liver and spleen
(3) Signs of marrow failure
= (pallor, petechiae, oral candida)
(4) Signs of infiltration
= (papilloedema, meningism, testicular enlargement, bone pain)
A 12-year-old girl presents to the GP with a sore throat. She has been feeling tired and run down recently and has noticed her gums bleed excessively when she brushes her teeth. Her mum has also noticed lots of bruises on her legs and arms that she can’t explain.
On examination, she looks pale, has a temperature of 37.2, and a heart rate of 100. Her throat is red and inflamed. On abdominal examination, there is cervical and inguinal lymphadenopathy and hepatosplenomegaly. You notice multiple petechiae on her legs.
What is the most important next step in management?
Urgent same day referral to paediatrics
20–30% have a poor-risk disease with poor-risk prognostic factors.
What are these poor prognostic factors?
(1) Age <1 year and >10 years
(2) Male sex
(3) White Cell Count >50 × 109/l
(4) CNS disease
(5) Poor cytogenetic features, such as t(9;22) = Philadelphia chromosome
(6) T-ALL – prognosis is worse than for B-ALL
(7) Incomplete/ slow response to therapy
