Please confirm topic selection

Are you sure you want to trigger topic in your Anconeus AI algorithm?

Please confirm action

You are done for today with this topic.

Would you like to start learning session with this topic items scheduled for future?

Review Question - QID 210372

In scope icon M 2 C
QID 210372 (Type "210372" in App Search)
A 59-year-old man presents to his primary care provider with fatigue. He has been seen in the emergency room 5 times in the past 2 years for acute alcohol intoxication. His past medical history is notable for diabetes mellitus and gout. He takes metformin, glyburide, and allopurinol. His temperature is 98.6°F (37°C), blood pressure is 130/85 mmHg, pulse is 86/min, and respirations are 16/min. He is in no acute distress. A positive fluid wave is noted. The dorsal surface of the tongue is depapillated, and there are erythematous swollen patches at the oral commissures. A complete blood count and iron studies are reported below:

Hemoglobin: 12.1 g/dL
Hematocrit: 36%
Leukocyte count: 6,000/mm^3 with normal differential
Platelet count: 170,000/mm^3

Mean corpuscular volume: 70 um^3
Iron: 250 ug/dL
Ferritin: 300 ng/mL
Total iron binding capacity: 200 mcg/dL^3

Which of the following findings is most likely to be seen in this patient?

Erythroblasts with mitochondrial iron deposits

51%

81/159

Erythrocytes lacking central pallor

9%

15/159

Erythrocytes with a ring of relative pallor

7%

11/159

Hypochromatic erythrocytes

25%

40/159

Inclusions of denatured hemoglobin

2%

3/159

Select Answer to see Preferred Response

bookmode logo Review TC In New Tab

The patient in this vignette is a chronic alcoholic with fatigue, glossitis, angular cheilitis, microcytic anemia, and increased free iron suggestive of sideroblastic anemia. Sideroblastic anemia is characterized by nucleated erythroblasts (sideroblasts) with peri-nuclear mitochondrial iron deposits.

Sideroblastic anemia is a form of microcytic anemia caused by a defect in erythropoiesis. Specifically, a disorder in the heme synthesis pathway allows iron to enter the mitochondria even though it cannot be incorporated into heme molecules. As a result, iron remains stuck in mitochondria, forming a ring around cell nuclei. This can be seen on a bone marrow aspirate as ringed sideroblasts, which are hypochromic nucleated erythroblasts with peri-nuclear basophilic iron deposits, representing iron deposits in peripheral mitochondria. Common causes of sideroblastic anemia include chronic alcoholism, drugs (e.g., isoniazid), and genetic mutations in the heme synthesis pathway.

Illustration A demonstrates the characteristic appearance of a ringed sideroblast. Note the perinuclear basophilic deposits which represent iron in peripheral mitochondria.

Incorrect Answers:
Answer 2: Erythrocytes lacking central pallor (spherocytes) are seen in hereditary spherocytosis. This is an autosomal dominant intrinsic hemolytic anemia caused by a defect in the erythrocyte membrane. It will often present with a normocytic hemolytic anemia in children.

Answer 3: Erythrocytes with a ring of relative pallor (target cells) are seen in thalassemias. There are different forms and severities of thalassemias that often present with severe microcytic anemia.

Answer 4: Hypochromatic erythrocytes (doughnut cells) are seen in iron deficiency anemia. This is an extremely common condition that may present with fatigue, pallor, tachycardia, glossitis, brittle nails, and microcytic anemia.

Answer 5: Inclusions of denatured hemoglobin (Heinz bodies) are seen in erythrocytes in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency, an X-linked genetic disorder causing an intrinsic hemolytic anemia.

Bullet Summary:
Sideroblastic anemia will present with nucleated erythroblasts (sideroblasts) with peri-nuclear mitochondrial iron deposits on a bone marrow aspirate.

ILLUSTRATIONS:
Authors
Rating
Please Rate Question Quality

3.8

  • star icon star icon star icon
  • star icon star icon star icon
  • star icon star icon star icon
  • star icon star icon star icon
  • star icon star icon star icon

(4)

Attach Treatment Poll
Treatment poll is required to gain more useful feedback from members.
Please enter Question Text
Please enter at least 2 unique options
Please enter at least 2 unique options
Please enter at least 2 unique options