Question 71#

Which of the following is a good prognostic factor in chronic lymphocytic leukaemia?

A. Female sex

Correct Answer A: 

Chronic lymphocytic leukaemia: prognostic factors:

Poor prognostic factors (median survival 3-5 years):

• Male sex
• Age > 70 years
• Lymphocyte count > 50
• Prolymphocytes comprising more than 10% of blood lymphocytes
• Lymphocyte doubling time < 12 months
• Raised LDH
• CD38 expression positive

Question 72#

What are the most common types of transformations seen in patients with polycythaemia rubra vera?

A. Myelodysplasia + chronic myeloid leukaemia

Correct Answer D: Polycythaemia rubra vera - around 5-15% progress to myelofibrosis or AML.

Polycythaemia rubra vera: management:
Polycythaemia rubra vera is a myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets.. It has peak incidence in the sixth decade, with typical features including hyperviscosity, pruritus and splenomegaly.

Management:

• Venesection - first line treatment
• Hydroxyurea -slight increased risk of secondary leukaemia
• Phosphorus-32 therapy

Prognosis:

• Thrombotic events are a significant cause of morbidity and mortality
• 5-15% of patients progress to myelofibrosis
• 5-15% of patients progress to acute leukaemia (risk increased with chemotherapy treatment)

Question 73#

A 54-year-old female is receiving a course of chemotherapy for breast cancer. She is experiencing troublesome vomiting which has not been helped by domperidone.

What is the most appropriate next management step?

A. Add an antihistamine

Correct Answer E: 

Chemotherapy side-effects: nausea and vomiting:
Nausea and vomiting are common side-effects of chemotherapy.

Risk factors for the development of symptoms include:

• Anxiety
• Age less than 50 years old
• Concurrent use of opioids
• The type of chemotherapy used

For patients at low-risk of symptoms then drugs such as metoclopramide may be used first-line. For high-risk patients then 5HT3 receptor antagonists such as ondansetron are often effective, especially if combined with dexamethasone.

Question 74#

Each one of the following is associated with iron-deficiency anaemia, except:

A. Atrophic glossitis

Correct Answer B: 

Iron deficiency anaemia:
Features:

• Koilonychia
• Atrophic glossitis
• Post-cricoid webs
• Angular stomatitis

Blood film: 

• Target cells
• 'Pencil' poikilocytes
• If combined with B12/folate deficiency a 'dimorphic' film occurs with mixed microcytic and macrocytic cells

Question 75#

A 50-year-old woman is investigated for weight loss and anaemia. She has no past medical history of note. 

Clinical examination reveals splenomegaly associated with pale conjunctivae. A full blood count is reported as follows:

• Hb 10.9 g/dl
• Platelets 702 * 10⁹/l
• WCC 56.6 * 10⁹/l
• Blood Film Leucocytosis noted. All stages of granulocyte maturation seen

Given the likely diagnosis, what is the most appropriate treatment?

A. Chlorambucil

Correct Answer E: 

Chronic myeloid leukaemia:

The Philadelphia chromosome is present in more than 95% of patients with chronic myeloid leukaemia (CML). It is due to a translocation between the long arm of chromosome 9 and 22 - t(9:22)(q34; q11). This results in part of the ABL proto-oncogene from chromosome 9 being fused with the BCR gene from chromosome 22. The resulting BCR-ABL gene codes for a fusion protein which has tyrosine kinase activity in excess of normal.

Presentation (40-50 years):

• Middle-age
• Anaemia, weight loss, abdo discomfort
• Splenomegaly may be marked
• Spectrum of myeloid cells seen in peripheral blood
• Decreased neutrophil alkaline phosphatase
• May undergo blast transformation (AML in 80%, ALL in 20%)

Management: 

• Imatinib is now considered first-line treatment
• Hydroxyurea
• Interferon-alpha
• Allogenic bone marrow transplant

Imatinib: 

• Inhibitor of the tyrosine kinase associated with the BCR-ABL defect
• Very high response rate in chronic phase CML