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MRCP Part 1 > Oncology / Haematology 2 > Flashcards

Oncology / Haematology 2 Flashcards

1
Q

A 40yo male is admitted with recurrent pancreatitis. A CT scan reveals no pancreatic mass, but evidence of widespread lymphadenopathy. Liver imaging shows a stricture in the common bile duct but no stones. He also has a history of parotiditis. What is the most likely diagnosis?

A

IgG4 disease

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2
Q

Why might IgG4-related disease be described as being analogous to Sarcoidosis?

A

In both diseases, diverse organ manifestations are linked by similar histopathological characteristics.

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3
Q

What may be used to assist in diagnosing IgG4 disease?

A

Raised concentrations of IgG4 in tissue and serum.

*Note: this can be helpful in diagnosing IgG4 disease, but neither is a specific diagnostic marker.

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4
Q

Which organ systems can be affected by IgG4-related disease?

A

Virtually every organ system:

  • Biliary tree
  • Salivary glands
  • Periorbital tissues
  • Kidneys
  • Lungs
  • Lymph nodes
  • Meninges
  • Aorta
  • Breast
  • Prostate
  • Thyroid
  • Pericardium
  • Skin
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5
Q

List some examples of IgG4-related diseases.

A
  • Riedel’s thyroiditis
  • Autoimmune pancreatitis
  • Mediastinal and Retroperitoneal fibrosis
  • Periaortitis / periarteritis / inflammatory aortic aneurysm
  • Kuttner’s tumour (submandibular glands) and Mikulicz Syndrome (salivary and lacrimal glands)
  • Possibly Sjogren’s and Primary Biliary Cirrhosis
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6
Q

A 26yo newly qualified nurse presents as she has developed a bilateral erythematous rash on both hands. She has recently emigrated from the Philippines and has no past medical history of note. A diagnosis of Contact Dermatitis is suspected. What is the most suitable test to identify the underlying cause?

A

Skin patch test

  • The skin patch test is useful in this situation as it may also identify irritants, not just allergens.
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7
Q

Describe how a Skin Prick Test is carried out.

A
  • Drops of diluted allergen are placed on the skin, after which the skin is pierced using a needle.
  • A large number of allergens can be tested in one session.
  • Normally includes a histamine (positive) and water (negative) control.
  • A wheal will typically develop if a patient has an allergy.
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8
Q

How long do you have to wait until the skin prick test can be interpreted?

A

15 minutes

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9
Q

Which allergies are the Skin Prick test useful for?

A
  • Food allergies

- Pollen

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10
Q

What is the principle behind the RAST (Radioallergosorbent test) test?

A

Determines the amount of IgE that reacts specifically with suspected or known allergens, eg. IgE to egg protein.

Results are given in grades from 0 (negative) to 6 (strongly positive)

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11
Q

Why might RAST be used instead of skin prick testing?

A

Blood tests (RAST) may be used when skin prick tests are not suitable, eg. if there is extensive eczema or if the patient is taking antihistamines.

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12
Q

Which allergies is the RAST test useful for?

A
  • Food allergies
  • Inhaled allergens (eg. pollen)
  • Wasp / bee venom
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13
Q

Which allergen test should be used to confirm a diagnosis of ?contact dermatitis?

A

Skin patch testing

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14
Q

How is Skin Patch testing carried out?

A
  • Around 30 - 40 allergens are placed on the back.
  • The patches are removed 48 hours later with the results being read by a Dermatologist after a further 48 hours.
  • Irritants may also be tested for in this way.
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15
Q

A 25yo female presents with recurrent sinopulmonary infections. What test is most likely to confirm a Primary Immunodeficiency?

A

IgG level

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16
Q

What is the most clinically significant Primary Immunodeficiency?

A

Common Variable Immunodeficiency (CVID).

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17
Q

What is the most common Primary Immunodeficiency?

A

IgA deficiency (but most are asymptomatic).

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18
Q

Define CVID (Common Variable Immunodeficiency).

A
  • The presence of hypogammaglobulinaemia of two or more immunoglobulin isotopes (low IgG, IgA or IgM)
  • Recurrent sinopulmonary infections
  • Impaired functional antibody responses.
  • However, IgG is more likely to be deficient than IgM.
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19
Q

What is the criteria for ‘impaired functional antibody responses’?

A
  • Absent isohaemagluttinins (eg. antibodies associated with blood transfusion reactions)
  • Poor responses to protein (diptheria, tetanus) or polysaccharide vaccines (S. pneumoniae), or both.
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20
Q

Which cells are likely to be absent in X-linked Bruton’s agammaglobulinaemia?

A

Mature B-cells.

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21
Q

A 45yo woman is diagnosed with Non-Hodgkin’s lymphoma.
She is a recovering alcoholic and has significant alcohol-related peripheral neuropathy.
Which chemotherapy agent should be avoided if possible, given her past history?

A

Vincristine: can cause (reversible) peripheral neuropathy

Inhibits formation of microtubules.

22
Q

A 34yo man who has Type 1 von Willebrand’s disease asks for advice. He is due to have a tooth extracted at the dentist next week. Which one of the following is the most appropriate management to reduce the risk of bleeding?

  • Mefanamic acid
  • Vitamin K
  • Desmopressin
  • Factor VIII concentrate
  • Factor VII concentrate
A

Desmopressin: raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells.

23
Q

Which disease is the most common inherited bleeding disorder?

A

Von Willebrand’s disease

24
Q

How are the majority of cases of von Willebrand disease inherited?

A

Autosomal Dominant

25
Q

List 2 symptoms which are commonly seen in von Willebrand disease, and 2 symptoms which are rare.

A

Common: Epistaxis, Menorrhagia

Rare: Haemarthroses, Muscle haematomas.

26
Q

What is the role of von Willebrand factor in normal physiology?

A
  • Promotes platelet adhesion to damaged endothelium
  • Carrier molecule for Factor VIII
  • vWF is a large glycoprotein which forms massive multimers up to 1,000,000 Da in size
27
Q

What is the difference between Types 1, 2 and 3 von Willebrand disease?

A

Type 1: partial reduction in vWF (80% of patients)

Type 2: abnormal form of vWF

Type 3: total lack of vWF (Autosomal Recessive)

28
Q

What might investigations yield if a patient has von Willebrand’s disease?

A
  • Prolonged bleeding time
  • APTT may be prolonged
  • Factor VIII levels may be moderately reduced
  • Defective platelet aggregation with ristocetin.
29
Q

Describe the management of a patient with von Willebrand’s disease.

A
  • Tranexamic acid for mild bleeding
  • Desmopressin (DDAVP) raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
  • Factor VIII concentrate.
30
Q

What is Type 2A von Willebrand’s disease caused by?

A

Type 2A: Caused by defective platelet adhesion due to decreased high molecular weight vWF multimers (i.e. the vWF protein is too small).

31
Q

What is Type 2B von Willebrand’s disease characterised by?

A

Type 2B: Characterised by an pathological increase of vWF-platelet interaction.

32
Q

What is Type 2M von Willebrand’s disease caused by?

A

Type 2M: caused by a decrease in vWF-plately interaction (not related to loss of high molecular weight multimers).

33
Q

What is Type 2N von Willebrand’s disease caused by?

A

Type 2N: Caused by abnormal binding of the vWF to Factor VIII.

34
Q

What is the correlation between symptomatic presentation and type of von Willebrand’s disease?

A
  • No clear correlation
  • Common themes amongst patients include:
    > Excessive mucocutaneous bleeding
    > Bruising in the absence of trauma
    > Menorrhagia in females
35
Q

Which is the most severe form of von Willebrand’s disease?

A

Type 3 = most severe form

Inherited as an Autosomal Recessive trait.

36
Q

Which of the following is least likely to cause a warm autoimmune haemolytic anaemia?

  • Mycoplasma infection
  • Methyldopa
  • Chronic lymphocytic leukaemia
  • Lymphoma
  • Systemic Lupus Erythematous
A

Mycoplasma infection
-> Mycoplasma infection causes a COLD autoimmune haemolytic anaemia.

SLE can rarely be associated with a mixed-type autoimmune haemolytic anaemia.

37
Q

How is Autoimmune haemolytic anaemia (AIHA) divided into ‘warm’ and ‘cold’ types?

A

AIHA can be divided into warm and cold types, according to at what temperature the antibodies best cause haemolysis.

38
Q

What causes AIHA (Autoimmune Haemolytic Anaemia)?

A
  • Idiopathic (most common)
  • Secondary to:
    > Lymphoproliferative disorder
    > Infection
    > Drugs
39
Q

How is AIHA (autoimmune haemolytic anaemia) identified?

A

Positive direct antiglobulin test (Coombs’ test)

40
Q

In Warm AIHA, which antibody is usually implicated?

A

Usually IgG

41
Q

In Warm AIHA, what temperature does haemolysis occur ‘best’ at?

A

Body temperature

42
Q

In Warm AIHA, where does haemolysis tend to occur?

A

In the extravascular sites eg, the spleen.

43
Q

What are the management options for Warm AIHA?

A
  • Steroids
  • Immunosuppression
  • Splenectomy
44
Q

What are the causes of Warm AIHA?

A
  • Autoimmune disease eg. SLE
  • Neoplasia eg. Lymphoma, CLL
  • Drugs eg. Methyldopa
45
Q

Which antibody is usually implicated in Cold AIHA?

A

Usually IgM

46
Q

In Cold AIHA, what temperature does haemolysis occur ‘best’ at?

A

4 degrees centigrade

47
Q

Describe the haemolysis seen in Cold AIHA.

A

Haemolysis is:

  • Mediated by complement
  • More commonly intravascular (as opposed to Warm AIHA, where haemolysis is extravascular).
48
Q

List 2 symptoms seen in a patient with Cold AIHA.

A
  • Raynauds

- Acrocyanosis (blue/purple colouring of the feet, caused by slow circulation)

49
Q

What are the causes of Cold AIHA?

A
  • Neoplasia eg. Lymphoma

- Infections eg. Mycoplasma, EBV

50
Q

Which disease can (rarely) be associated with a mixed-type Autoimmune Haemolytic Anaemia?

A

Systemic Lupus Erythematous

MRCP Part 1 (16 decks)

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