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MRCP Part 1 > Oncology / Haematology 1 > Flashcards

Oncology / Haematology 1 Flashcards

1
Q

A patient is started on Cyclophosphamide for vasculitis associated with Wegener’s granulomatosis. Which of the following is characteristically associated with Cyclophosphamide?

  • Haemorrhagic cystitis
  • Cardiomyopathy
  • Ototoxicity
  • Alopecia
  • Weight gain
A

Haemorrhagic cystitis

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2
Q

What is the mechanism of action of cyclophosphamide?

A

Alkylating agent - causes cross-linking in DNA

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3
Q

List 3 adverse effects of Cyclophosphamide.

A
  • Haemorrhagic cystitis
  • Myelosuppression
  • Transitional cell carcinoma
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4
Q

Name 2 cytotoxic antibiotics.

A
  • Bleomycin

- Doxorubicin

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5
Q

What is the mechanism of Bleomycin (a cytotoxic antibiotic)?

A

Degrades pre-formed DNA

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6
Q

What is one adverse effect of Bleomycin?

A

Lung fibrosis

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7
Q

What is the mechanism of action of Doxorubicin?

A

Stabilizes DNA-topoisomerase II complex; inhibits DNA and RNA synthesis.

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8
Q

What is one adverse effect of Doxorubicin?

A

Cardiomyopathy

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9
Q

Name 4 Antimetabolites.

A
  • Methotrexate
  • Fluorouracil (5-FU)
  • 6-Mercaptopurine
  • Cytarabine
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10
Q

What is the mechanism of action of methotrexate?

What are 3 adverse effects associated with Methotrexate?

A

Inhibits dihydrofolate reductase and thymidylate synthesis

Adverse effects:

  • Myelosuppression
  • Liver fibrosis
  • Lung fibrosis
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11
Q

What is the mechanism of action of Fluorouracil (5-FU)?

Adverse effects of 5-FU?

A

Pyrimidine analogues inducing cell cycle arrest and apoptosis by blocking thymidylate synthase (works during S phase).

Adverse effects:

  • Myelosuppression
  • Mucositis
  • Dermatitis
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12
Q

What is the mechanism of action of 6-Mercaptopurine?

Adverse effects?

A

Purine analogue that is activated by HGPRTase, decreasing purine synthesis.

Adverse effects:
- Myelosuppression

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13
Q

What is the mechanism of action of Cytarabine?

Adverse effects of this drug?

A

Pyrimidine antagonist. Interferes with DNA synthesis specifically at the S-phase of the cell cycle and inhibits DNA polymerase.

Adverse effects:

  • Myelosuppression
  • Ataxia
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14
Q

List 3 drugs which act on microtubules.

A
  • Vincristine
  • Vinblastine
  • Docetaxel
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15
Q

For Vincristine, describe:

i) The mechanism of action
ii) Adverse effects

A

i) Inhibits formation of microtubules

ii)
- Peripheral neuropathy (reversible)
- Paralytic ileum

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16
Q

For Vinblastine, describe:

i) the mechanism of action
ii) Adverse effects

A

i) Inhibits formation of microtubules

ii) Myelosuppression

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17
Q

For Docetaxel, describe:

i) The mechanism of action
ii) Adverse effects

A

i) Prevents microtubule depolymerisation and disassembly, decreasing free tubulin.
ii) Adverse effects: Neutropenia

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18
Q

Give an example of a topoisomerase inhibitor.
What is its mechanism of action?
Adverse effects?

A
  • Irinotecan
  • Inhibits topoisomerase I which prevents relaxation of supercoiled DNA
  • Adverse effects: myelosuppression
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19
Q

For Cisplatin, describe:

i) Mechanism of action
ii) Adverse effects

A

i) Causes cross-linking in DNA

ii) Adverse effects:
- Ototoxicity
- Peripheral neuropathy
- Hypomagnesaemia

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20
Q

For Hydroxyurea (Hydroxycarbamide) describe:

i) Mechanism of action
ii) Adverse effects

A

i) Inhibits ribonucleotide reductase, decreasing DNA syntheis
ii) Adverse effects: Myelosupression.

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21
Q

Which of the following is most associated with thymomas?

  • Myelodysplasia
  • Thrombocytopenia
  • Acute myeloid leukaemia
  • Acute lymphoblastic leukaemia
  • Red cell aplasia
A

Red cell aplasia

22
Q

What is a thymoma?

A

The most common tumour of the anterior mediastinum.

Usually detected between the 6th and 7th decades of life.

23
Q

Which diseases is a Thymoma associated with?

A
  • Myaesthenia Gravis (30-40% of patients have a thymoma)
  • Red cell aplasia
  • Dermatomyositis
  • also: SLE, SIADH
24
Q

How does a thymoma cause death?

A
  • Compression of airway

- Cardiac tamponade

25
Q

Which of the following causes of Primary Immunodeficiency is due to a defect in B-cell function?

  • Di George syndrome
  • Chediak-Higashi syndrome
  • Common variable immunodeficiency
  • Chronic granulomatous disease
  • Wiskott-Aldrich syndrome
A

Common variable immunodeficiency.

26
Q

List 3 Primary Immunodeficiency disorders which are due to Neutrophil disorders.

A
  • Chronic Granulomatous Disease
  • Chediak-Higashi syndrome
  • Leukocyte adhesion deficiency
27
Q

What is the underlying defect in Chronic Granulomatous disease?

A

Lack of NADPH oxidase reduces the ability of phagocytes to produce reactive oxygen species.

28
Q

Give a clinical overview of a patient with Chronic Granulomatous disease.

A
  • Recurrent pneumonias and abscesses, particularly due to catalase-positive bacteria (eg. S. aureus) and fungi (e.g. Aspergillus).
  • Negative Nitroblue-tetrazolium test
  • Abnormal dihydrohodamine flow cytometry test
29
Q

What is the underlying defect in Chediak-Higashi syndrome?

A

Microtubule polymerisation defect leads to a decrease in phagocytosis.

30
Q

Give a clinical overview of a patient with Chediak-Higashi syndrome.

A
  • Affected children have ‘partial albinism’ and peripheral neuropathy.
  • Recurrent bacterial infections are seen
  • Giant granules in neutrophils and platelets
31
Q

What is the underlying defect in Leukocyte adhesion deficiency?

A

Defect of LFA-1 integrin (CD18) protein on Neutrophils.

32
Q

Give a clinic overview of a patient with Leukocyte adhesion deficiency.

A
  • Recurrent bacterial infections
  • Delay in umbilical cord sloughing may be seen
  • Absence of neutrophils/pus at sites of infection
33
Q

List 3 B-cell disorders associated with Primary Immunodeficiency.

A
  • Common variable immunodeficiency
  • Bruton’s (X-linked) congenital agammaglobulinaemia
  • Selective Immunoglobulin A Deficiency
34
Q

For ‘Common Variable Immunodeficiency’, describe:

i) the underlying defect
ii) clinical presentation

A

i) Many varying causes

ii) Hypogammaglobulinaemia is seen. May predispose to autoimmune disorders and lymphoma

35
Q

For ‘Bruton’s (X-linked) congenital agammaglobulinaemia, describe the underlying defect.

A

Defect in Bruton’s tyrosine kinase (BTK) gene that leads to a severe block in B cell development

36
Q

Describe the clinical presentation of someone with Bruton’s congenital agammaglobulinaemia.

A
  • X-linked recessive
  • Recurrent bacterial infections are seen
  • Absence of B-cells with reduced immunoglobulins of all classes.
37
Q

What is the underlying defect associated with Selective Immunoglobulin A deficiency?

A

Maturation defect in B cells.

38
Q

Describe the clinical presentation of someone with Selective Immunoglobulin A deficiency.

A
  • The most common primary antibody deficiency.
  • Recurrent sinus and respiratory infections
  • Associated with Coeliac disease and may cause false negative coeliac antibody screen
  • Severe reactions to blood transfusions may occur (anti-IgA antibodies -> anaphylaxis).
39
Q

Which T-cell disorder is associated with Primary Immunodeficiency?

A

DiGeorge syndrome

40
Q

What is the underlying defect in a patient with DiGeorge syndrome?

A
  • 22q11.2 deletion

- Failure to develop 3rd and 4th pharyngeal pouches

41
Q

List some common features of a patient with DiGeorge syndrome.

A
  • Congenital heart disease (eg. tetralogy of Fallot)
  • Learning difficulties
  • Hypocalcaemia
  • Recurrent viral/fungal diseases
  • Cleft palate
42
Q

List 4 combined B- and T-cell disorders.

A
  • Severe combined immunodeficiency
  • Ataxic telangiectasia
    Wiskott-Aldrich syndrome
  • Hyper IgM syndromes
43
Q

What is the underlying defect that causes Severe Combined Immunodeficiency?

A
  • Many varying causes
  • Most common (X-linked) due to defect in the common gamma chain, a protein used in the receptors for IL-2 and other interleukins.
  • Other causes include Adenosine deaminase deficiency.
44
Q

How might a patient with Severe Combined Immunodeficiency present?

A
  • Recurrent infections due to viruses, bacteria and fungi
  • Reduced T-cell receptor excision circles
  • Stem cell transplantation may be successful
45
Q

What is the underlying defect that causes Ataxic telangiectasia?

A

Defect in DNA repair enzymes

46
Q

How is Ataxic telangiectasia inherited. List some features of the disease.

A

Autosomal recessive inheritance.

  • Cerebellar ataxia
  • Telangiectasia (spider angiomas)
  • Recurrent chest infections
  • 10% risk of developing malignancy, lymphoma or leukaemia
47
Q

What is the underlying defect in a patient with Wiskott-Aldrich syndrome?

A

Defect in WASP gene

48
Q

How is Wiskott-Aldrich syndrome inherited?

A

X-linked recessive.

49
Q

List some features of Wiskott-Aldrich syndrome.

A
  • Recurrent bacterial infections
  • Eczema
  • Thrombocytopenia
  • Low IgM levels
  • Increased risk of autoimmune disorders and malignancy.
50
Q

Which defect is responsible for Hyper IgM syndromes?

A

Mutations in the CD40 gene.

51
Q

List some presenting features of a patient with a Hyper IgM syndrome.

A
  • Infection / pneumocystis
  • Pneumonia
  • Hepatitis
  • Diarrhoea.

MRCP Part 1 (16 decks)

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