Practice questions Anemia II
Practice questions
Anemia II
© Jun Wang, MD, PhD
Abbreviations:
|
Hb: hemoglobin |
HCT: hematocrit |
|
MCV: Mean Corpuscular Volume |
MCH: Mean Corpuscular Hemoglobin |
|
RDW: Red Cell Distribution Width |
TIBC: total iron binding capacity |
1. Use this case for the next six questions. A 6-year-old boy presents to the clinic with fatigue, irritability, and episodes of pain in his hands and feet over the past few months. The pain episodes last for several hours and seem to occur more frequently recently. Physical examination reveals pale skin and mucosa and mild scleral icterus. Splenomegaly is noted on abdominal palpation. Laboratory studies are listed below:
Reticulocyte count: 10% (1-2%)
White blood cell count: 12,000/µL (5,000-14,500/µL)
Platelet count: 400,000/µL (150,000-450,000/µL)
Indirect bilirubin: 1.2 mg/dL (0.2-0.8 mg/dL)
An image of his peripheral blood smear is shown. What is the most appropriate next step to confirm the diagnosis?
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Image credit: Ed Uthman from Houston, TX, USA, CC BY 2.0 <https://creativecommons.org/licenses/by/2.0>, via Wikimedia Commons
B. Chromosomal breakage tests
C. Hemoglobin electrophoresis
D. Osmotic fragility test
E. Serum ferritin
2. A 6-year-old boy presents to the clinic with fatigue,
irritability, and episodes of pain in his hands and feet over the past few
months. The pain episodes last for several hours and seem to occur more
frequently recently. Physical examination reveals pale skin and mucosa and mild
scleral icterus. Splenomegaly is noted on abdominal palpation. Laboratory
studies are listed below:
Reticulocyte count: 10% (1-2%)
White blood cell count: 12,000/µL (5,000-14,500/µL)
Platelet count: 400,000/µL (150,000-450,000/µL)
Indirect bilirubin: 1.2 mg/dL (0.2-0.8 mg/dL)
An image of his peripheral blood smear is shown. What is the most likely results of his hemoglobin electrophoresis?
Image credit: Ed Uthman from Houston, TX, USA, CC BY 2.0 <https://creativecommons.org/licenses/by/2.0>, via Wikimedia Commons
|
Hb (%) |
HbA |
HbA2 |
HbF |
HbS |
HbC |
|
A. |
0 |
2 |
4 |
96 |
0 |
|
B. |
0 |
2 |
4 |
45 |
49 |
|
C. |
0 |
10 |
80 |
0 |
10 |
|
D. |
54 |
4 |
2 |
0 |
40 |
|
E. |
90 |
0 |
10 |
0 |
0 |
|
F. |
96 |
2 |
2 |
0 |
0 |
3. A 6-year-old boy presents to the clinic with fatigue, irritability, and episodes of pain in his hands and feet over the past few months. The pain episodes last for several hours and seem to occur more frequently recently. Physical examination reveals pale skin and mucosa and mild scleral icterus. Splenomegaly is noted on abdominal palpation. Laboratory studies are listed below:
Reticulocyte count: 10% (1-2%)
White blood cell count: 12,000/µL (5,000-14,500/µL)
Platelet count: 400,000/µL (150,000-450,000/µL)
Indirect bilirubin: 1.2 mg/dL (0.2-0.8 mg/dL)
An image of his peripheral blood smear is shown. Hemoglobin electrophoresis reveals absent HbA, slightly increased HbF, and predominantly HbS. What is causing the morphological features of his RBCs?
Image credit: Ed Uthman from Houston, TX, USA, CC BY 2.0 <https://creativecommons.org/licenses/by/2.0>, via Wikimedia Commons
B. Lack of hemoglobin alpha chain
C. Lack of hemoglobin beta chain
D. Loss of unstable membrane
E. Polymerization of hemoglobin
4. A 6-year-old boy presents to the clinic with fatigue, irritability, and episodes of pain in his hands and feet over the past few months. The pain episodes last for several hours and seem to occur more frequently recently. Physical examination reveals pale skin and mucosa and mild scleral icterus. Splenomegaly is noted on abdominal palpation. Laboratory studies are listed below:
Reticulocyte count: 10% (1-2%)
White blood cell count: 12,000/µL (5,000-14,500/µL)
Platelet count: 400,000/µL (150,000-450,000/µL)
Indirect bilirubin: 1.2 mg/dL (0.2-0.8 mg/dL)
An image of his peripheral blood smear is shown. Hemoglobin electrophoresis reveals absent HbA, slightly increased HbF, and predominantly HbS. What genetic abnormality is most likely seen in this patient?
Image credit: Ed Uthman from Houston, TX, USA, CC BY 2.0 <https://creativecommons.org/licenses/by/2.0>, via Wikimedia Commons
B. Deletion of 1 beta chain
C. Glutamate to lysine substitution at codon 6 of 1 beta chain
D. Glutamate to lysine substitution at codon 26 of 1 beta chain
E. Glutamate to Valine substitution at codon 6 of 2 beta chain
5. A 6-year-old boy presents to the clinic with fatigue, irritability, and episodes of pain in his hands and feet over the past few months. The pain episodes last for several hours and seem to occur more frequently recently. Physical examination reveals pale skin and mucosa and mild scleral icterus. Splenomegaly is noted on abdominal palpation. Laboratory studies are listed below:
Reticulocyte count: 10% (1-2%)
White blood cell count: 12,000/µL (5,000-14,500/µL)
Platelet count: 400,000/µL (150,000-450,000/µL)
Indirect bilirubin: 1.2 mg/dL (0.2-0.8 mg/dL)
An image of his peripheral blood smear is shown. Hemoglobin electrophoresis reveals absent HbA, slightly increased HbF, and predominantly HbS. What is the diagnosis?
Image credit: Ed Uthman from Houston, TX, USA, CC BY 2.0 <https://creativecommons.org/licenses/by/2.0>, via Wikimedia Commons
B. Beta thalassemia major
C. Hemoglobin C disease
D. Sickle cell anemia
E. Sickle cell trait
6. A 6-year-old boy presents to the clinic with fatigue, irritability, and episodes of pain in his hands and feet over the past few months. The pain episodes last for several hours and seem to occur more frequently recently. Physical examination reveals pale skin and mucosa and mild scleral icterus. Splenomegaly is noted on abdominal palpation. Laboratory studies are listed below:
Reticulocyte count: 10% (1-2%)
White blood cell count: 12,000/µL (5,000-14,500/µL)
Platelet count: 400,000/µL (150,000-450,000/µL)
Indirect bilirubin: 1.2 mg/dL (0.2-0.8 mg/dL)
An image of his peripheral blood smear is shown. Hemoglobin electrophoresis reveals absent HbA, slightly increased HbF, and predominantly HbS. What is the cause of his elevated bilirubin?
Image credit: Ed Uthman from Houston, TX, USA, CC BY 2.0 <https://creativecommons.org/licenses/by/2.0>, via Wikimedia Commons
B. Liver disease
C. Oxidative stress
D. RBC membrane protein cross-linking
E. Surface complement activity
7. Use this case for the next four questions. A 10-year-old boy presents to the emergency department with severe pain in his lower back and legs for the past 24 hours. He has a history of multiple similar episodes, typically triggered by cold weather or dehydration. His past medical history is significant for recurrent episodes of pain and multiple hospital admissions. On examination, he is in acute distress, with a temperature of 38.2°C (100.8°F), heart rate of 110/min, and blood pressure of 100/60 mmHg. He has scleral icterus, and his spleen is palpable 3 cm below the costal margin. Laboratory tests show the following:
White blood cell count: 15,000/µL (4,500-13,500/µL)
Platelet count: 450,000/µL (150,000-450,000/µL)
Reticulocyte count: 12% (0.5-2.5%)
Lactate dehydrogenase (LDH): 600 U/L (140-280 U/L)
Total bilirubin: 4.0 mg/dL (0.3-1.2 mg/dL)
(Image credit: Dr Graham Beards, CC BY-SA 3.0 <https://creativecommons.org/licenses/by-sa/3.0>, via Wikimedia Commons)
An image of his peripheral blood smear is shown. Which test should be performed to confirm the diagnosis?
B. Chromosomal breakage tests
C. Hemoglobin electrophoresis
D. Osmotic fragility test
E. Serum ferritin
8. A 10-year-old boy presents to the emergency department with
severe pain in his lower back and legs for the past 24 hours. He has a history
of multiple similar episodes, typically triggered by cold weather or
dehydration. His past medical history is significant for recurrent episodes of
pain and multiple hospital admissions. On examination, he is in acute distress,
with a temperature of 38.2°C (100.8°F), heart rate of 110/min, and blood
pressure of 100/60 mmHg. He has scleral icterus, and his spleen is palpable 3
cm below the costal margin.Laboratory tests show the following:
White blood cell count: 15,000/µL (4,500-13,500/µL)
Platelet count: 450,000/µL (150,000-450,000/µL)
Reticulocyte count: 12% (0.5-2.5%)
Lactate dehydrogenase (LDH): 600 U/L (140-280 U/L)
Total bilirubin: 4.0 mg/dL (0.3-1.2 mg/dL)
(Image credit: Dr Graham Beards, CC BY-SA 3.0 <https://creativecommons.org/licenses/by-sa/3.0>, via Wikimedia Commons)
A peripheral blood smear is shown. Hemoglobin electrophoresis reveals absent HbA, slightly increased HbF, and predominantly HbS. Which of the following is the most likely underlying genetic mutation in this patient?
B. Glutamate to lysine substitution at codon 6 of 1 beta chain
C. Glutamate to lysine substitution at codon 26 of 1 beta chain
D. Glutamate to Valine substitution at codon 6 of 1 beta chain
E. Glutamate to Valine substitution at codon 6 of 2 beta chain
9. A 10-year-old boy presents to the emergency department with
severe pain in his lower back and legs for the past 24 hours. He has a history
of multiple similar episodes, typically triggered by cold weather or
dehydration. His past medical history is significant for recurrent episodes of
pain and multiple hospital admissions. On examination, he is in acute distress,
with a temperature of 38.2°C (100.8°F), heart rate of 110/min, and blood
pressure of 100/60 mmHg. He has scleral icterus, and his spleen is palpable 3
cm below the costal margin.Laboratory tests show the following:
White blood cell count: 15,000/µL (4,500-13,500/µL)
Platelet count: 450,000/µL (150,000-450,000/µL)
Reticulocyte count: 12% (0.5-2.5%)
Lactate dehydrogenase (LDH): 600 U/L (140-280 U/L)
Total bilirubin: 4.0 mg/dL (0.3-1.2 mg/dL)
(Image credit: Dr Graham Beards, CC BY-SA 3.0 <https://creativecommons.org/licenses/by-sa/3.0>, via Wikimedia Commons)
A peripheral blood smear is shown. Hemoglobin electrophoresis reveals absent HbA, slightly increased HbF, and predominantly HbS. What is most likely causing his pains?
B. Bacterial infection
C. Platelet thrombosis
D. Red cell aggregation
E. Trauma
10. A 10-year-old boy presents to the emergency department with
severe pain in his lower back and legs for the past 24 hours. He has a history
of multiple similar episodes, typically triggered by cold weather or
dehydration. His past medical history is significant for recurrent episodes of
pain and multiple hospital admissions. On examination, he is in acute distress,
with a temperature of 38.2°C (100.8°F), heart rate of 110/min, and blood
pressure of 100/60 mmHg. He has scleral icterus, and his spleen is palpable 3
cm below the costal margin. Laboratory tests show the following:
White blood cell count: 15,000/µL (4,500-13,500/µL)
Platelet count: 450,000/µL (150,000-450,000/µL)
Reticulocyte count: 12% (0.5-2.5%)
Lactate dehydrogenase (LDH): 600 U/L (140-280 U/L)
Total bilirubin: 4.0 mg/dL (0.3-1.2 mg/dL)
(Image credit: Dr Graham Beards, CC BY-SA 3.0 <https://creativecommons.org/licenses/by-sa/3.0>, via Wikimedia Commons)
A peripheral blood smear is shown. Hemoglobin electrophoresis reveals absent HbA, slightly increased HbF, and predominantly HbS. What is the diagnosis?
B. Aplastic crisis
C. Hemolytic crisis
D. Pain crisis
E. Sequestration crisis
11. Use this case for the next two questions. A 5-year-old boy presents to the emergency department with sudden onset of weakness, pallor, and abdominal pain. His parents report that he has been unusually lethargic and has had decreased appetite for the past day. Physical examination reveals a tachycardic and hypotensive child with a markedly enlarged spleen palpable in the left upper quadrant. Laboratory studies show:
White cell count: 11 x 10³/mm³ (4.5–10.5 x 10³/mm³)
Reticulocyte count: 18% (0.5-2.5%)
Platelet count: 100 x 10³/mm³ (194–364 x 10³/mm³)
Indirect bilirubin: 1.2 mg/dL (0.2-0.8 mg/dL)
LDH: 500 IU/L (150–300 IU/L)
A peripheral blood smear is shown. What is the most likely diagnosis?
(Image credit: Gregory Kato, CC BY-SA 4.0 <https://creativecommons.org/licenses/by-sa/4.0>, via Wikimedia Commons)
B. Aplastic crisis
C. Hemolytic crisis
D. Pain crisis
E. Sequestration crisis
12. A 5-year-old boy presents to the emergency department with sudden onset of weakness, pallor, and abdominal pain. His parents report that he has been unusually lethargic and has had decreased appetite for the past day. Physical examination reveals a tachycardic and hypotensive child with a markedly enlarged spleen palpable in the left upper quadrant. Laboratory studies show:
Hemoglobin: 5 g/dL (previously 9 g/dL)White cell count: 11 x 10³/mm³ (4.5–10.5 x 10³/mm³)
Reticulocyte count: 18% (0.5-2.5%)
Platelet count: 100 x 10³/mm³ (194–364 x 10³/mm³)
Indirect bilirubin: 1.2 mg/dL (0.2-0.8 mg/dL)
LDH: 500 IU/L (150–300 IU/L)
A peripheral blood smear is shown. What is the most likely Hb electrophoresis result?
(Image credit: Gregory Kato, CC BY-SA 4.0 <https://creativecommons.org/licenses/by-sa/4.0>, via Wikimedia Commons)
|
Hb (%) |
HbA |
HbA2 |
HbF |
HbS |
HbC |
|
A. |
0 |
2 |
4 |
96 |
0 |
|
B. |
0 |
2 |
4 |
45 |
49 |
|
C. |
0 |
10 |
80 |
0 |
10 |
|
D. |
54 |
4 |
2 |
0 |
40 |
|
E. |
90 |
0 |
10 |
0 |
0 |
|
F. |
96 |
2 |
2 |
0 |
0 |
13. Use this case for the next question. A 16-year-old girl presents to the emergency department with fever, chest pain, and shortness of breath. She reports that these symptoms started abruptly two days ago. She has a history of sickle cell anemia. She has a temperature of 101. 3°F, heart rate of 110/min, respiratory rate of 28/min, and oxygen saturation at 88% on room air. Physical examination reveals tachypnea, decreased breath sounds and dullness to percussion over the right lower lung field. Laboratory studies reveal:
White blood cell count: 15 x 10³/mm³ (4.8–10.1 x 10³/mm³)
Reticulocyte count: 10% (0.5-2.5%)
Platelet count: 250,000/mm³ (194–364 x 10³/mm³)
A chest X-ray shows a new infiltrate in the right lower lobe. An image of her peripheral blood smear is shown. What is the diagnosis?
(Image credit: Paulo Henrique Orlandi Mourao, CC BY-SA 4.0 <https://creativecommons.org/licenses/by-sa/4.0>, via Wikimedia Commons)
B. Aplastic crisis
C. Hemolytic crisis
D. Pain crisis
E. Sequestration crisis
14. Use this case for the next two questions. A 22-year-old male athlete presents to the clinic with persistent hematuria for a week. He has been training intensively for an upcoming marathon. He reports no history of trauma, dysuria, or flank pain. He has no significant past medical history and takes no medications. He has a few paternal family members with sickle cell disease. Physical examination is unremarkable. His CBC, PT and aPTT are within normal ranges. Urinalysis reveals numerous red blood cells but no casts or signs of infection. Hemoglobin electrophoresis reveals 60% HbA, 38% HbS, and 2% HbA2. What is the most likely diagnosis?
B. Diabetic nephropathy
C. Hemoglobin C disease
D. Sickle cell disease
E. Sickle cell trait
15. A 22-year-old male athlete presents to the clinic with persistent hematuria for a week. He has been training intensively for an upcoming marathon. He reports no history of trauma, dysuria, or flank pain. He has no significant past medical history and takes no medications. He has a few paternal family members with sickle cell disease. Physical examination is unremarkable. His CBC, PT and aPTT are within normal ranges. Urinalysis reveals numerous red blood cells but no casts or signs of infection. Hemoglobin electrophoresis reveals 60% HbA, 38% HbS, and 2% HbA2. What is causing his hematuria?
B. Interstitial inflammation of kidney
C. Papillary necrosis
D. Urinary tract inflammation
E. Vitamin K deficiency
16. Use this case for the next three questions. A 25-year-old man presents to the clinic with fatigue and mild jaundice for 6 months. His past medical history is unremarkable. Physical examination reveals mild splenomegaly. Laboratory studies show the following:
Mean corpuscular volume (MCV): 85 fL (80-100 fL)
Reticulocyte count: 4% (0.5-2.5%)
Direct bilirubin: 0.5 mg/dL (< 0.5 mg/dL)
An image of his peripheral blood smear is shown. Which test should be performed to confirm the diagnosis?
(Image credit: isis325)
B. Enzymatic activity of G6PD
C. Eosin-5-maleimide (EMA) binding assay
D. Hemoglobin electrophoresis
E. Osmotic fragility test
17. A 25-year-old man presents to the clinic with fatigue and mild jaundice for 6 months. His past medical history is unremarkable. Physical examination reveals mild splenomegaly. Laboratory studies show the following:
Hemoglobin: 10 g/dL (10.9–15.7 g/dL)Mean corpuscular volume (MCV): 85 fL (80-100 fL)
Reticulocyte count: 4% (0.5-2.5%)
Direct bilirubin: 0.5 mg/dL (< 0.5 mg/dL)
An image of his peripheral blood smear is shown. Hemoglobin electrophoresis reveals 0% HbA, 0% HbS, 98% HbC and 2% HbA2. What genetic abnormality is most likely seen in this patient?
(Image credit: isis325)
B. Glutamate to lysine substitution at codon 6 of 2 beta chain
C. Glutamate to lysine substitution at codon 26 of 2 beta chain
D. Glutamate to Valine substitution at codon 6 of 1 beta chain
E. Glutamate to Valine substitution at codon 6 of 2 beta chain
18. A 25-year-old man presents to the clinic with fatigue and mild jaundice for 6 months. His past medical history is unremarkable. Physical examination reveals mild splenomegaly. Laboratory studies show the following:
Hemoglobin: 10 g/dL (10.9–15.7 g/dL)Mean corpuscular volume (MCV): 85 fL (80-100 fL)
Reticulocyte count: 4% (0.5-2.5%)
Direct bilirubin: 0.5 mg/dL (< 0.5 mg/dL)
An image of his peripheral blood smear is shown. Hemoglobin electrophoresis reveals 0% HbA, 0% HbS, 98% HbC and 2% HbA2. What is the diagnosis?
(Image credit: isis325)
B. Hemoglobin C disease
C. Sickle cell anemia
D. Sickle cell trait
E. Thalassemia beta major
19. Use this case for the next three questions. A 20-year-old woman presents to the emergency department with severe pain in her lower back and joints that began abruptly two days ago. She reports similar episodes in the past, often triggered by dehydration or cold weather. She appears in acute distress but afebrile. Physical examination reveal mild scleral icterus. Non-tender spleen is palpated 3 cm below left costal margin. Her laboratory studies are listed below:
MCV: 85 fL (80-100 fL)
Reticulocyte count: 8% (0.5-2.5%)
Direct bilirubin: 0.8 mg/dL (<0.3 mg/dL)
Lactate dehydrogenase (LDH): 250 IU/L (42–166 IU/L)
An image of her peripheral blood smear is shown. What is the most likely Hb electrophoresis result?
(Image credit: isis325)
|
Hb (%) |
HbA |
HbA2 |
HbF |
HbS |
HbC |
|
A. |
0 |
2 |
4 |
96 |
0 |
|
B. |
0 |
2 |
4 |
45 |
49 |
|
C. |
0 |
10 |
80 |
0 |
10 |
|
D. |
54 |
4 |
2 |
0 |
40 |
|
E. |
90 |
0 |
10 |
0 |
0 |
|
F. |
96 |
2 |
2 |
0 |
0 |
20. A 20-year-old woman presents to the emergency department with severe pain in her lower back and joints that began abruptly two days ago. She reports similar episodes in the past, often triggered by dehydration or cold weather. She appears in acute distress but afebrile. Physical examination reveal mild scleral icterus. Non-tender spleen is palpated 3 cm below left costal margin. Her laboratory studies are listed below:
MCV: 85 fL (80-100 fL)
Reticulocyte count: 8% (0.5-2.5%)
Direct bilirubin: 0.8 mg/dL (<0.3 mg/dL)
Lactate dehydrogenase (LDH): 250 IU/L (42–166 IU/L)
An image of her peripheral blood smear is shown. What genetic abnormality is most likely seen?
(Image credit: isis325)
B. Glutamate to lysine substitution at codon 6 of 1 beta chain, and Glutamate to Valine substitution at codon 6 of the other beta chain
C. Glutamate to lysine substitution at codon 6 of 2 beta chain
D. Glutamate to Valine substitution at codon 6 of 1 beta chain
E. Glutamate to Valine substitution at codon 6 of 2 beta chain
21. A 20-year-old woman presents to the emergency department with severe pain in her lower back and joints that began abruptly two days ago. She reports similar episodes in the past, often triggered by dehydration or cold weather. She appears in acute distress but afebrile. Physical examination reveal mild scleral icterus. Non-tender spleen is palpated 3 cm below left costal margin. Her laboratory studies are listed below:
MCV: 85 fL (80-100 fL)
Reticulocyte count: 8% (0.5-2.5%)
Direct bilirubin: 0.8 mg/dL (<0.3 mg/dL)
Lactate dehydrogenase (LDH): 250 IU/L (42–166 IU/L)
An image of her peripheral blood smear is shown. What is the diagnosis?
(Image credit: isis325)
B. Hemoglobin C disease
C. Hemoglobin SC disease
D. Sickle cell trait
E. Thalassemia beta major
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