ATI Hematologic System

ATI Hematologic System Related

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A 14-year-old Syrian male with beta thalassemia major has relocated to your community as a refugee. He has been receiving chronic transfusion therapy in Turkey for the past 3 years. On his first visit, you notice that his height is below the fifth percentile. He has skin discoloration and hepatosplenomegaly. His mother reports they have not had regular access to chelation therapy. Laboratory testing shows a serum ferritin of 6,200 ng/mL. A cardiac MRI shows grossly normal cardiac function but a T2* value of 9 ms. What is the most likely cause of his short stature?

  • A. Lack of regular blood transfusion causing growth failure
  • B. Cirrhosis and liver failure
  • C. Ineffective erythropoiesis and chronic anemia
  • D. Growth hormone deficiency due to iron deposition in the pituitary
Correct Answer: D

Rationale: The correct answer is D: Growth hormone deficiency due to iron deposition in the pituitary. Iron overload in patients with beta thalassemia major can lead to iron deposition in various organs, including the pituitary gland, impairing its function. This can result in growth hormone deficiency, leading to short stature. In this case, the patient's history of chronic transfusions and high serum ferritin level indicate iron overload, which can affect the pituitary gland. Choices A, B, and C are incorrect because growth failure in beta thalassemia major is primarily attributed to endocrine complications such as growth hormone deficiency, rather than lack of transfusions, cirrhosis, or ineffective erythropoiesis.

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A newborn infant develops jaundice on day of life 2. Labs are drawn, and she has a hemoglobin of 7.4 g/dL with a reticulocyte count of 8%. Upon peripheral blood smear review, she is found to have bizarre red cell forms with significant polkilocytosis. Although her parents have normal blood counts, on review of their peripheral blood smears, they both have a moderate number of ovalocytes. Which of the following is the most likely cause of the infant's red cell findings?

  • A. She has an autosomal dominant ankyrin mutation from one of her parents causing hereditary spherocytosis.
  • B. She has inherited band 3 variants from each parent and will likely need a splenectomy after she turns 5 years old.
  • C. She has inherited an alpha-spectrin mutation from both of her parents and may experience an improvement in her anemia over time.
  • D. She has inherited a PKLR variant from each parent, and enzyme testing will be consistent with her diagnosis of pyruvate kinase deficiency.
Correct Answer: C

Rationale: The correct answer is C because the infant's red cell findings of ovalocytes and polikilocytosis are consistent with hereditary elliptocytosis, which is caused by mutations in genes encoding spectrin. In this scenario, the inheritance pattern aligns with the infant receiving an alpha-spectrin mutation from both parents, leading to the observed erythrocyte abnormalities. This condition may improve over time due to compensatory mechanisms.

Choice A (autosomal dominant ankyrin mutation causing hereditary spherocytosis) is incorrect because the clinical presentation and red cell morphology do not support a diagnosis of hereditary spherocytosis.

Choice B (inherited band 3 variants and need for splenectomy) is incorrect as the infant's condition does not match the characteristics of hereditary spherocytosis requiring splenectomy.

Choice D (PKLR variant and pyruvate kinase deficiency) is incorrect as the infant's red cell morphology is not indicative

A patient is presented with weakness of legs, arms, trunk, tingling and numbness that progressively worsens. Peripheral blood smear shows macrocytic anemia. Which of the following is not the factor causing this condition?

  • A. Chronic atrophic gastritis
  • B. Ileal resection
  • C. Tapeworm infestation
  • D. Alcoholism
Correct Answer: C

Rationale: The correct answer is C: Tapeworm infestation. The symptoms described suggest vitamin B12 deficiency, which can lead to macrocytic anemia. Chronic atrophic gastritis can cause decreased intrinsic factor production, leading to impaired B12 absorption. Ileal resection can also affect B12 absorption as it is the site where B12 is absorbed. Alcoholism can lead to poor dietary intake and impaired B12 absorption. However, tapeworm infestation does not directly affect B12 absorption and would not be a likely cause of the described symptoms.

A patient receiving blood begins complaining of severe chest pain and a feeling of warmth. What should the nurse do first?

  • A. Call the physician.
  • B. Administer diuretics as ordered.
  • C. Discontinue the blood transfusion.
  • D. Assess vital signs and cardiovascular status.
Correct Answer: C

Rationale: The correct first step is to discontinue the blood transfusion (Choice C) because the patient is experiencing symptoms of a transfusion reaction, such as chest pain and warmth. Stopping the transfusion is crucial to prevent further harm. Calling the physician (Choice A) can be done after discontinuing the transfusion. Administering diuretics (Choice B) is not appropriate for this situation. Assessing vital signs and cardiovascular status (Choice D) should be done after stopping the transfusion to monitor the patient's condition.

Which of the following will be present in chronic bacterial infection?

  • A. Neutrophilia
  • B. Basophilia
  • C. Lymphocytosis
  • D. Monocytosis
Correct Answer: D

Rationale: The correct answer is D: Monocytosis. In chronic bacterial infections, monocytosis occurs due to the increased presence of monocytes in response to the persistent infection. Monocytes differentiate into macrophages to help eliminate the bacteria. Neutrophilia (A) is more common in acute bacterial infections. Basophilia (B) is associated with conditions like chronic myeloid leukemia, not bacterial infections. Lymphocytosis (C) is often seen in viral infections or chronic inflammatory conditions, not specifically in chronic bacterial infections.

The nurse is explaining the role of red blood cells with oxygen transport in the body with a nursing student. Which term should the nurse use to describe hemoglobin that has given up its oxygen to the body's cells?

  • A. Reduced
  • B. Detached
  • C. Oxyhemoglobin
  • D. Hypoxyhemoglobin
Correct Answer: A

Rationale: The correct term to describe hemoglobin that has given up its oxygen to the body's cells is "Reduced" (Choice A). This term refers to hemoglobin that has released its oxygen molecules and is now in a deoxygenated state.
Rationale:
1. Hemoglobin binds to oxygen in the lungs (forming oxyhemoglobin).
2. When hemoglobin reaches the body's cells, it releases oxygen for cellular use.
3. Once hemoglobin releases oxygen, it becomes deoxygenated or "Reduced".
Summary:
- Choice B (Detached) does not accurately describe the process of oxygen release by hemoglobin.
- Choice C (Oxyhemoglobin) refers to hemoglobin bound to oxygen, not hemoglobin that has released oxygen.
- Choice D (Hypoxyhemoglobin) would refer to hemoglobin that has a reduced oxygen level, not hemoglobin that has given up its oxygen to the cells.

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