A 4-year-old girl with a history of recurrent epistaxis and easy bruising is referred to you for evaluation. She is found to have a prolonged PTT and a factor VIII level that is less than 1%. Both parents have a history of excessive bleeding. She is admitted with a severe episode of epistaxis, and your colleague orders 40 IU/kg of recombinant factor VIII. Her epistaxis resolves initially but within an hour starts again at the same severity as before. What is the best next step?

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Anatomy of Hematologic System Questions

Question 1 of 5

A 4-year-old girl with a history of recurrent epistaxis and easy bruising is referred to you for evaluation. She is found to have a prolonged PTT and a factor VIII level that is less than 1%. Both parents have a history of excessive bleeding. She is admitted with a severe episode of epistaxis, and your colleague orders 40 IU/kg of recombinant factor VIII. Her epistaxis resolves initially but within an hour starts again at the same severity as before. What is the best next step?

Correct Answer: A

Rationale: The correct answer is A: Infuse a von Willebrand factor concentrate. In this scenario, the 4-year-old girl has a history of recurrent epistaxis and easy bruising, indicative of a bleeding disorder. The prolonged PTT and factor VIII level less than 1% suggest a deficiency in von Willebrand factor (VWF) or factor VIII. When the initial dose of recombinant factor VIII did not completely resolve the epistaxis, it indicates a possible deficiency in VWF activity. Therefore, the best next step is to infuse a von Willebrand factor concentrate to address the VWF deficiency, which should help control the bleeding. Summary: - Option B (Give another dose of recombinant factor VIII concentrate) is not the best choice because the initial dose did not fully resolve the bleeding, indicating a different factor may be deficient. - Option C (Call otorhinolaryngology to pack her nose) addresses the symptom but does not

Question 2 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 3 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 4 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.

Question 5 of 5

The nurse is assessing a client experiencing anemia. Which laboratory findings will the nurse expect for this client? (Select all that apply.)

Correct Answer: b

Rationale: Correct Answer: B - Decreased red blood cell count Rationale: 1. Anemia is defined by a decrease in red blood cells, causing decreased oxygen-carrying capacity. 2. A decreased red blood cell count is a key indicator of anemia. 3. Other choices are incorrect: A. Increased hematocrit is not expected in anemia, as it indicates a higher concentration of red blood cells. C. Decreased serum iron may be seen in iron-deficiency anemia but is not a universal finding in all types of anemia. D. Decreased hemoglobin is a common finding in anemia but is not as specific as a decreased red blood cell count.

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