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Hamilton 2020 Questions > Hematology > Flashcards

Hematology Flashcards

1
Q 
Maisie is a previously well 3-year old girl presenting to ER with: Fever 39.2oC, Pallor, Jaundice, Tachycardia
-She was previously treated for 4 days for neonatal jaundice
with phototherapy 
-Otherwise well
 On assessment you find she has: 
Fever 39.2oC
Jaundice
Pallor
Tachycardia 168 bpm 
Ejection systolic murmur 
Splenomegaly - ~ 7cm
Hgb 46 (118 – 137 g/L)
WBC 19.8 (5.5 – 12 x 109/L)
Platelets 458 (150 – 400 x 109/L)
Bilirubin 177 (2 – 17 micromol/L)

Urinalysis
Negative for bilirubin & haemoglobin

Which type of jaundice is this?

a) Conjugated (direct)
b) Unconjugated (indirect)

A

b) Unconjugated (indirect)

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2
Q 
Maisie is a previously well 3-year old girl presenting to ER with: Fever 39.2oC, Pallor, Jaundice, Tachycardia
-She was previously treated for 4 days for neonatal jaundice
with phototherapy 
-Otherwise well
 On assessment you find she has: 
Fever 39.2oC
Jaundice
Pallor
Tachycardia 168 bpm 
Ejection systolic murmur 
Splenomegaly - ~ 7cm
Hgb 46 (118 – 137 g/L)
WBC 19.8 (5.5 – 12 x 109/L)
Platelets 458 (150 – 400 x 109/L)
Bilirubin 177 (2 – 17 micromol/L)

Which of these initial investigations is LEAST likely to be useful in determining the cause of her jaundice & anaemia?

a) Direct antibody test (DAT)
b) Serum haptoglobin
c) Blood group
d) Blood smear examination

A
  1. Blood group
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3
Q 
Maisie is a previously well 3-year old girl presenting to ER with: Fever 39.2oC, Pallor, Jaundice, Tachycardia
-She was previously treated for 4 days for neonatal jaundice
with phototherapy 
-Otherwise well
 On assessment you find she has: 
Fever 39.2oC
Jaundice
Pallor
Tachycardia 168 bpm 
Ejection systolic murmur 
Splenomegaly - ~ 7cm
Hgb 46 (118 – 137 g/L)
WBC 19.8 (5.5 – 12 x 109/L)
Platelets 458 (150 – 400 x 109/L)
Bilirubin 177 (2 – 17 micromol/L)
DAT Negative
Haptoglobin Normal
Blood smear: small RBCs with no central clearing

Given the information you have so far, what is the most likely reason for Maisie’s jaundice?

  1. Autoimmune haemolytic anaemia
  2. Hypersplenism
  3. G6PD deficiency
  4. Hereditary spherocytosis
A
  1. Hereditary spherocytosis
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4
Q

Is hereditary spherocytosis an example of:

  1. Intravascular haemolysis?
  2. Extravascular haemolysis?
A
  1. Extravascular haemolysis?
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5
Q

Clinically, you decide a patient needs a blood transfusion. Her blood group is B negative. Which of these blood groups would be a compatible donor?

  1. AB negative
  2. B positive
  3. A negative
  4. O positive
  5. O negative
A
  1. O negative
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6
Q

Which is the commonest transfusion-associated adverse event?

  1. Transfusion-related acute lung injury (TRALI)
  2. Transfusion-associated circulatory overload (TACO)
  3. Serious allergic reaction
  4. CMV infection
A
  1. Transfusion-associated circulatory overload (TACO)
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7
Q

What is the estimated chance of contracting HIV from a blood transfusion in Canada?

  1. 1 in 500,000 transfusions
  2. 1 in 1.3 million transfusions
  3. 1 in 4.5 million transfusions
  4. 1 in7.8 million transfusions
  5. 1 in 21.4 million transfusions
A
  1. 1 in 21.4 million transfusions
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8
Q

At what age should a patient have a routine splenectomy for hereditary spherocytosis?

  1. 3 years
  2. 5 years
  3. 10 years
  4. Routine splenectomy not recommended
A
  1. Routine splenectomy not recommended
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9
Q

Which of these statements about post-splenectomy infection is TRUE?

  1. Patients are only at increased risk of infection with encapsulated bacteria
  2. If patients are fully immunised against H. influenza, S. pneumonia and N. meningitidis, they do not need prophylactic antibiotic treatment
  3. Antibiotic prophylaxis is not needed in sickle cell anaemia patients
  4. Antibiotic prophylaxis is recommended for a minimum of 2 years post-splenectomy
A
  1. Antibiotic prophylaxis is recommended for a minimum of 2 years post-splenectomy
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10
Q

2-year old Dwayne presents with recurrent abdominal pain. No cause has previously been found on clinical examination or investigation.
He has previously had episodes of painful, swollen hands
On examination, he is in pain, febrile (38.5oC), tachycardic (158 bpm), with blood pressure 118/75. Saturations are 93% in room air
He has pallor, but no jaundice, lymphadenopathy, nor cyanosis
Abdomen is globally tender, with a 3-cm spleen
Routine bloodwork is taken:

Hb 64 (123 – 146 g/L)
WBC 16.4 (5.1 – 11.2 x 109/L)
Platelets 358 (150 – 400 x 109/L)
Haematocrit 18% (38–46%)
MCV 89 (81–91fL)
Creatinine 72 (21 – 54 micromol/L)
BUN 6.2 (1.3 – 5.5 mmol/L)
Bilirubin 13 (1 – 17 micromol/L)

With which diagnosis are these results and history most compatible?

  1. Sickle Cell Anaemia
  2. Chronic iron deficiency anaemia
  3. G6PD deficiency
  4. Hereditary Spherocytosis
  5. Hemolytic Uremic Syndrome
A
  1. Sickle Cell Anaemia
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11
Q

You suspect sickle cell disease. What is the most EFFECTIVE way of establishing your diagnosis in the ED?

  1. Hemoglobin electrophoresis
  2. Sickledex screen
  3. Osmotic fragility test
  4. Blood smear examination
A
  1. Sickledex screen
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12
Q

Which of these is a positive Sickledex screen?

  1. cloudy
  2. clear
A
  1. cloudy
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13
Q

What is the molecular cause of Sickle Cell Anaemia?

  1. Single point mutation in the alpha globin gene
  2. Single point mutation in the beta globin gene
  3. Deletion of the alpha globin gene
  4. Deletion of the beta globin gene
A
  1. Single point mutation in the beta globin gene
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14
Q

What is the mechanism behind sickling of red blood cells?

  1. Formation of SSSS tetramers within the RBC
  2. Oxidative damage to Hb molecules producing methaemoglobin
  3. Polymerisation of haemoglobin to form strands
  4. Hypoxic damage to the cell membrane leading to deformation
A
  1. Polymerisation of haemoglobin to form strands
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15
Q

Which statement Is TRUE regarding acute pain management in Sickle cell disease?

  1. Acetaminophen is not a useful adjunct in acute pain crisis
  2. Parents can have opiates at home for acute pain management
  3. NSAIDs are contraindicated due to renal toxicity
  4. Opiate nurse-controlled infusions are preferable to opiate PCA (patient-controlled analgesia) in acute crisis due to the risk of overdose
A
  1. Parents can have opiates at home for acute pain management
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16
Q

Which of these statements about transfusion therapy in Sickle Cell disease is CORRECT?

  1. Donor blood should be screened, and be negative for sickle cell trait
  2. Transfusion should not be given in splenic sequestration as it will increase consumption
  3. The goal in chronic transfusion therapy for SCD is to maintain Hb level above 100 g/L
  4. The goal in chronic transfusion therapy for SCD is to maintain HbS levels below 30%
A
  1. The goal in chronic transfusion therapy for SCD is to maintain HbS levels below 30%
17
Q

What is the mechanism for Hydroxyurea’s activity in sickle cell disease?

  1. It reduces the tendency of HbS to sickle
  2. It increases the production of Hb F
  3. It increases the production of HbA2
  4. It reduces RBC production and reduces blood viscosity
A
  1. It increases the production of Hb F
18
Q

Keenan is a 6-year old boy requiring hernia repair. History suggest he has always been an easy bruiser and has a history of sporadic nosebleeds.
His mother has mild, well-controlled Lupus
His bloodwork shows normal INR, prolonged PTT, normal fibrinogen.
Which of these is the MOST appropriate first test to perform?
1. Antinuclear antibody screen (ANA)
2. Factor VIII level
3. Von Willebrand antigen/activity
4. 50:50 mixing study
5. Factor IX level

A
  1. 50:50 mixing study
19
Q

Keenan is a 6-year old boy requiring hernia repair. History suggest he has always been an easy bruiser and has a history of sporadic nosebleeds.
His mother has mild, well-controlled Lupus
His bloodwork shows normal INR, prolonged PTT, normal fibrinogen.

50:50 mixing study:
Control 27.4 (22 – 37 seconds)
Patient 69.5 (22 – 37 seconds)
Control:Patient 28.2 (22 – 37 seconds)

What does this result imply?

  1. He has lupus
  2. He has a factor deficiency
  3. He has an inhibitor
  4. He has haemophilia A
A
  1. He has a factor deficiency
20
Q

Keenan is a 6-year old boy requiring hernia repair. History suggest he has always been an easy bruiser and has a history of sporadic nosebleeds.
His mother has mild, well-controlled Lupus
His bloodwork shows normal INR, prolonged PTT, normal fibrinogen.

50:50 mixing study:
Control 27.4 (22 – 37 seconds)
Patient 69.5 (22 – 37 seconds)
Control:Patient 28.2 (22 – 37 seconds)

Which of these is unlikely to be a possible diagnosis for Keenan?

  1. Afibrinogenaemia
  2. Factor VIII deficiency
  3. Factor IX deficiency
  4. Von Willebrand disease
A
  1. Afibrinogenaemia
21
Q

Keenan is a 6-year old boy requiring hernia repair. History suggest he has always been an easy bruiser and has a history of sporadic nosebleeds.
His mother has mild, well-controlled Lupus
His bloodwork shows normal INR, prolonged PTT, normal fibrinogen.

Further bloodwork shows Factor VIII level = 0.07 (0.5 – 1.27 U/L)
He is diagnosed with Haemophilia A

What severity level of Haemophilia does he have?

  1. Mild
  2. Moderate
  3. Severe
A
  1. Mild
22
Q

Keenan is a 6-year old boy requiring hernia repair. History suggest he has always been an easy bruiser and has a history of sporadic nosebleeds.
His mother has mild, well-controlled Lupus
His bloodwork shows normal INR, prolonged PTT, normal fibrinogen.

Further bloodwork shows Factor VIII level = 0.07 (0.5 – 1.27 U/L)
He is diagnosed with Haemophilia A
What should the next step of management be?
a) PRN treatment with FVIII concentrates
b) Prophylactic treatment with FVIII concentrates
c) DDAVP challenge
d) PRN treatment with antifibrinolytics (e.g. tranexamic acid)

A

c) DDAVP challenge

23
Q

What is the mechanism of action of DDAVP (desmopressin) in Haemophilia A?

  1. Release of von Willebrand factor from endothelium
  2. Increased production of FVIII in liver
  3. Strengthening of fibrin-fibrin crosslinks
  4. Increased activation of FVIII by Thrombin
A
  1. Release of von Willebrand factor from endothelium
24
Q

Aliceis a previously well 12-year old girl
Develops widespread bruising and petechiae
Recently recovered from a heavy cold. Mom gave her ibuprofen and acetaminophen
Mom has history of heavy periods but no significant other bleeding problems
Normal growth (75th centile for height and weight)
Bilateral cervical lymphadenopathy (small shotty nodes) Extensive bruising over legs, arms and trunk
Petechiae over hard palate
Loose cough, no crackles audible in chest
She has a CBC taken: platelets, atypical lymphocytes on smear, otherwise unremarkable

What is Alice’s most likely diagnosis?

  1. Aplastic anaemia
  2. Acute leukaemia
  3. Acute immune thrombocytopoenia
  4. Chronic immune thrombocytopoenic purpura
  5. Henoch-Schonlein purpura
A
  1. Acute immune thrombocytopoenia
25
Q

Which of these would NOT be a reasonable first-line management for acute ITP?

  1. Observation
  2. Platelet transfusion
  3. Intravenous immunoglobulin
  4. Corticosteroids
  5. Anti-D immune globulin
A
  1. Platelet transfusion
26
Q

What is the goal of giving treatment for acute ITP?

  1. To shorten the duration of thrombocytopoenia
  2. To raise the platelet count to over 20 x 109/L
  3. To reduce the risk of severe bleeding
  4. To reassure parents
A
  1. To reduce the risk of severe bleeding
27
Q

Which of these is NOT a recognised serious side effect of IVIG treatment?

  1. Acute pancreatitis
  2. Aseptic meningitis
  3. Haemolytic anaemia
  4. Anaphylactoid reaction
A
  1. Acute pancreatitis
28
Q

How long must childhood ITP continue before being considered Chronic ITP?

  1. 3 months
  2. 6 months
  3. 12 months
  4. 24 months
A
  1. 12 months

Hamilton 2020 Questions (29 decks)

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