Acute Lymphoblastic Leukaemia

Question 1

What is Acute Lymphoblastic Leukaemia (ALL)?

Answer 1

A malignant disease of primitive lymphoid cells (lymphoblasts) where cells do not mature, staying in blast form due to arrested maturation

Question 2

What is the most common cancer in childhood?

Answer 2

Acute Lymphoblastic Leukaemia (ALL), peaking at 2-4 years

Question 3

What genetic abnormality is associated with ALL?

Answer 3

The Philadelphia chromosome (t(9:22) translocation) is found in 30% of adults and 3-5% of children with ALL

Question 4

Which genetic syndrome increases the risk of ALL?

Answer 4

Down syndrome

Question 5

What type of lymphocyte is usually involved in ALL?

Answer 5

B-lymphocytes

Question 6

How does ALL cause pancytopenia?

Answer 6

Excessive proliferation of malignant lymphoblasts replaces normal bone marrow cells

Question 7

Name three effects of marrow failure in ALL

Answer 7

Anaemia, infections, and bleeding

Question 8

What extra-medullary sites can ALL affect?

Answer 8

The CNS and testis

Question 9

What are some complications of high white cell counts in ALL?

Answer 9

Obstruction of circulation and superior vena cava (SVC) obstruction

Question 10

What findings on a blood film are characteristic of ALL?

Answer 10

(1) Abnormal cells (‘blasts’) -immature lymphoid cells
(2) Large size
(3) High nuclear-to-cytoplasmic ratio
(4) Prominent nucleolus

Question 11

What is the diagnostic threshold for blasts in acute leukaemia?

Answer 11

≥20% blasts in the peripheral blood or bone marrow

Question 12

Which tests are performed on a bone marrow aspirate in ALL?

Answer 12

(1) Morphology
(2) Immunophenotyping
(3) Cytogenetics

Question 13

What is the primary treatment approach for ALL?

Answer 13

Multi-agent chemotherapy lasting 2-3 years, including CNS-directed treatment and immunotherapy for specific subsets

Question 14

What is a Hickman line used for in ALL management?

Answer 14

Long-term central venous access during treatment

Question 15

What are the major complications of the disease itself in ALL?

Answer 15

(1) Anaemia
(2) Neutropenia (leading to severe infections)
(3) Thrombocytopenia (causing bleeding)

Question 16

What is the empirical treatment for neutropenic fever in ALL?

Answer 16

Broad-spectrum antibiotics, particularly covering gram-negative bacteria

Question 17

What is Tumour Lysis Syndrome, and when does it occur in ALL?

Answer 17

A life-threatening condition during the first course of treatment caused by rapid cell breakdown

Question 18

What are the potential late complications of ALL treatment?

Answer 18

Loss of fertility and cardiomyopathy

Question 19

Name a common fungal infection risk in prolonged neutropenia

Answer 19

Pneumocystis jirovecii pneumonia (PJP)

Question 20

What preventive treatment is given for PJP in ALL?

Answer 20

Prophylactic therapy against Pneumocystis jirovecii

Question 21

Complications of treatment for Acute Lymphoblastic Leukaemia is what?

Answer 21

(1) Hair loss
(2) Liver and renal dysfunction
(3) Tumour lysis syndrome (during the first course of treatment)
(4) Infection

Question 22

What are the clinical features of acute lymphocytic leukaemia (ALL)?

Answer 22

(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)

Question 23

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?

Answer 23

Urgent same day referral to paediatrics

Question 24

20–30% have a poor-risk disease with poor-risk prognostic factors.
What are these poor prognostic factors?

Answer 24

(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

Question 25

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?

Answer 25

Hyperdiploid blast cells

Question 26

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?

Answer 26

Raised serum phosphate + potassium High serum uric acid low serum calcium Describing Tumour lysis syndrome

Question 27

Blast cell predominance indicates what

Answer 27

diagnosis for Acute Lymphoblastic Leukaemia

Question 28

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?

Answer 28

Down syndrome = mentions fatigue so can't be thrombocytopenia

Question 29

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?

Answer 29

(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

Question 30

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?

Answer 30

(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

Question 31

Which chemotherapy drugs are commonly used in combination for treating acute lymphoblastic leukaemia (ALL), and what is their purpose in treatment?

Answer 31

(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

Question 32

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?

Answer 32

Full blood count

Question 33

When is t(12:21) the correct genetic abnormality when the patient is diagnosed with ALL?

Answer 33

young age (paediatrics)

Question 34

If blast cells are mentioned, what diseases does this colorate with?

Answer 34

Acute myeloid leukaemia (AML) Acute lymphoblastic leukaemia (ALL)

Question 35

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?

Answer 35

ALL typically presents in children under 6 years of age with features of marrow infiltration and lymphadenopathy

Question 36

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?

Answer 36

Leukaemic lymphoblastic cells

Question 37

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?

Answer 37

Aggressive hydration and allopurinol treatment