You have a new patient consult in clinic this morning. The referral packet includes the newborn screen report, which is flagged abnormal hemoglobinopathy screen, F, A, Bart's, refer to hematology, and a complete blood count done at 4 years of age with a hemoglobin of 10 g/dL and an MCV of 68. The pediatrician has informed the parents the child has some form of alpha thalassemia. The older brother had the same newborn screen results but had a normal complete blood count when checked. The mother wants to know why her second child has an abnormal complete blood count when she and her husband do not have any blood problems. How would you respond to the child's mother?

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Anatomy of Hematologic System Questions

Question 1 of 5

You have a new patient consult in clinic this morning. The referral packet includes the newborn screen report, which is flagged abnormal hemoglobinopathy screen, F, A, Bart's, refer to hematology, and a complete blood count done at 4 years of age with a hemoglobin of 10 g/dL and an MCV of 68. The pediatrician has informed the parents the child has some form of alpha thalassemia. The older brother had the same newborn screen results but had a normal complete blood count when checked. The mother wants to know why her second child has an abnormal complete blood count when she and her husband do not have any blood problems. How would you respond to the child's mother?

Correct Answer: A

Rationale: The correct answer is A because both parents are silent carriers, each passing a deleted alpha globin allele to their child, resulting in a trans-deletion genotype alpha thalassemia trait. This explanation aligns with the child's abnormal complete blood count and the family history. Choice B is incorrect because it states that the mother has cis deletion alpha thalassemia, which is not supported by the information provided. Choice C is incorrect as it suggests a new spontaneous mutation causing alpha thalassemia in the child, which is not consistent with the genetic inheritance pattern described. Choice D is incorrect because it claims both parents carry cis deletions in the alpha globin gene cluster, which contradicts the scenario where the parents are silent carriers.

Question 2 of 5

A 14-year-old male patient is diagnosed with very high risk acute lymphoblastic leukemia and is likely going to require an allogeneic hematopoietic stem cell transplant to cure his leukemia. Prior to going to transplant, he is likely to require multiple blood transfusions. Which of the following products or component modifications is the best way to prevent him from developing alloimmunization due to anti-HLA antibodies prior to transplant?

Correct Answer: D

Rationale: The correct answer is D, Leukoreduced blood products. Leukoreduction removes white blood cells from the blood product, reducing the risk of alloimmunization due to anti-HLA antibodies. White blood cells are a major source of HLA antigens that can stimulate the immune system to produce antibodies. This process helps prevent the development of alloimmunization, which is crucial for patients undergoing stem cell transplant. A: Frozen RBCs - Freezing does not affect the risk of alloimmunization. B: Volume-reduced blood products - Volume reduction does not specifically target white blood cells or HLA antigens. C: Irradiation of all blood products - Irradiation is used to prevent graft-versus-host disease, not specifically to prevent alloimmunization.

Question 3 of 5

A 20-month-old otherwise healthy male presents late for his 18-month well child check. During his first year of life, he took iron-fortified formula and had a point-of-care hemoglobin (Hgb) of 12 g/dL at his 1-year well child check. His mother reports that he is a picky eater but loves milk and has recently become obsessive about chewing the corners of his cardboard books. Physical examination is normal except for a flow murmur. Which combination of laboratory test results listed below would most likely characterize this patient?

Correct Answer: A

Rationale: The correct answer is A because it reflects iron deficiency anemia in a 20-month-old child who is a picky eater and has pica behavior. The low hemoglobin level of 8.7 g/dL indicates anemia. The MCV of 60 fL suggests microcytic anemia, typical of iron deficiency. The serum ferritin level of 2 ng/mL confirms low iron stores. Choice B is incorrect as the hemoglobin level is normal, MCV is higher, and serum ferritin is not indicative of iron deficiency anemia. Choice C is incorrect as the hemoglobin level is higher, and the MCV is not consistent with iron deficiency anemia. Choice D is incorrect as the hemoglobin level is higher, MCV is too high for iron deficiency anemia, and the serum ferritin level does not support iron deficiency.

Question 4 of 5

A 12-year-old patient has been referred to you following complete resection with clean margins of a high-grade malignant peripheral nerve sheath tumor of the shoulder region. The tumor measured approximately 4 cm in greatest dimension. A CT scan of the chest and a bone scan were within normal limits. The patient does not have evidence of neurofibromatosis type 1 (NF1). Which of the following treatment approaches would you recommend?

Correct Answer: D

Rationale: The correct answer is D: Observation. In this case, the patient has undergone complete resection of the tumor with clean margins and there is no evidence of metastasis or neurofibromatosis type 1. Given these factors, observation is the most appropriate approach as there is no indication for adjuvant chemotherapy or radiotherapy. Chemotherapy with doxorubicin and ifosfamide (Choice A) is typically reserved for cases with high-risk features such as incomplete resection or metastasis. Radiotherapy (Choice B) may be considered in certain cases, but in this scenario where surgery was curative and there is no evidence of spread, it is not necessary. Chemotherapy plus radiotherapy (Choice C) may be overly aggressive and carry unnecessary risks for a patient who has already had successful surgery. Thus, observation is the most appropriate approach in this situation.

Question 5 of 5

What is the nurse's priority when caring for a client who just completed a bone marrow aspiration and biopsy?

Correct Answer: C

Rationale: The correct answer is C: Check the pressure dressing frequently for signs of excessive or active bleeding. This is the priority because post bone marrow aspiration and biopsy, there is a risk of bleeding due to the procedure. By checking the dressing, the nurse can assess for any signs of excessive bleeding or hematoma formation, which are crucial to prevent complications. A: Teaching the client to avoid activity is important but not the priority immediately post-procedure. B: Administering NSAIDs may not be appropriate as they can increase the risk of bleeding. D: Reporting the lab results is important but not the priority over ensuring immediate post-procedure safety.

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