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
A 5-year-old boy is admitted to the paediatric haematology ward for treatment of acute lymphoblastic leukaemia (ALL). He is due to receive chemotherapy.
What is a good prognostic factor in ALL?
Hyperdiploid blast cells
A 3-year-old girl is undergoing chemotherapy treatment for acute lymphoblastic leukaemia (ALL). A few days into her first course of treatment she develops severe nausea and vomiting, muscle weakness and paraesthesia.
What biochemistry feature is thsi describing?
Raised serum phosphate + potassium
High serum uric acid
low serum calcium
Describing Tumour lysis syndrome
Blast cell predominance indicates what
diagnosis for Acute Lymphoblastic Leukaemia
A 4-year-old girl with a specific genetic condition comes to the GP with her mother. The mother is concerned about progressive fatigue in her daughter and has noticed that there are lots of small patches of ‘blood spots’ on her thighs. The mother also states that her daughter has had a fever for the last 10 days.
What genetic condition is most associated with the most likely diagnosis? Why?
Down syndrome
= mentions fatigue so can’t be thrombocytopenia
A 4-year-old girl with a specific genetic condition comes to the GP with her mother. The mother is concerned about progressive fatigue in her daughter and has noticed that there are lots of small patches of ‘blood spots’ on her thighs. The mother also states that her daughter has had a fever for the last 10 days
What in the above statement indicates that this 4-year girl has ALL?
(1) Progressive fatigue
= Indicates anaemia due to marrow failure
(2) Petechiae (‘blood spots’)
= Suggests thrombocytopenia from reduced platelet production
(3) Fever for 10 days
= Often due to infections or leukemia-driven immune dysfunction
A 3-year-old male patient presents with a 1-month history of lethargy and abnormal bruising. On physical examination, there is conjunctival pallor and multiple petechiae and ecchymoses on the trunk and limbs. There is cervical lymphadenopathy and moderate hepatosplenomegaly.
What from the above statement indicates that this 3-year-old has ALL?
(1) Lethargy and pallor
= Reflecting anaemia
(2) Petechiae, ecchymoses, and bruising = Indicating thrombocytopenia
(3) Lymphadenopathy and hepatosplenomegaly
= Typical findings in ALL due to infiltration of lymphoblasts
Which chemotherapy drugs are commonly used in combination for treating acute lymphoblastic leukaemia (ALL), and what is their purpose in treatment?
(1) vincristine
(2) cyclophosphamide
(3) doxorubicin
(4) prednisolone
= These drugs work together to target and kill leukemia cells at various stages of the cell cycle
A 3-year-old girl presents to the general practitioner with a 2-week history of fever and fatigue. She has presented to A&E 4 times in the last 6 months with recurrent infections. On examination, there is widespread painless lymphadenopathy and splenomegaly. In addition, there are petechiae on her forearms.
What is the first step in this patients mangement?
Full blood count
When is t(12:21) the correct genetic abnormality when the patient is diagnosed with ALL?
young age (paediatrics)
If blast cells are mentioned, what diseases does this colorate with?
Acute myeloid leukaemia (AML)
Acute lymphoblastic leukaemia (ALL)
A 52-year-old male patient presents to the general practitioner with a 4-week history of weight loss and malaise. He also reports discomfort in the left upper abdomen and abdominal fullness. There is no past medical history noted. He denies any recent travel.
On physical examination, a mass arises from the left upper quadrant, extending to the right iliac fossa. The mass has a palpable notch and a dull percussion note
Why wouldn’t Leukaemic lymphoblastic cells be found?
ALL typically presents in children under 6 years of age with features of marrow infiltration and lymphadenopathy
A 3-year-old male patient presents with a 1-month history of lethargy and abnormal bruising.
On physical examination, conjunctival pallor, multiple petechiae, and ecchymoses were found on the trunk and limbs. Cervicalpatosplenomegaly were also present lymphadenopathy and moderate he.
What blood film finding would be consistent with the most likely diagnosis?
Leukaemic lymphoblastic cells
A 45-year-old man with newly diagnosed acute lymphoblastic leukaemia presents to the emergency department with fatigue and lethargy. Labs reveal elevated uric acid and potassium levels, and a CT scan reveals widespread lymphadenopathy.
What is the most appropriate next step in management?
Aggressive hydration and allopurinol treatment