ATI RN
Anatomy of Hematologic System Questions
Question 1 of 5
A 2-year-old girl has a diagnosis of overall stage IV favorable histology Wilms' tumor with pulmonary metastases and local stage III disease due to finding positive lymph nodes. After she completes 6 weeks of vincristine/dactinomycin/doxorubicin (DD4A) chemotherapy, restaging shows complete resolution of some but not all lung nodules. Tumor genetic testing reveals combined loss of heterozygosity for 1p and 16q. Which of the following would be the most appropriate treatment plan?
Correct Answer: D
Rationale: The correct answer is D because the patient has residual lung nodules after initial chemotherapy, indicating a need for intensified treatment. Adding cyclophosphamide and etoposide to the chemotherapy regimen can improve outcomes in patients with combined loss of heterozygosity for 1p and 16q. Radiation to both the lungs and flank targets all remaining disease sites effectively. Choice A is incorrect because it does not address the need for intensified treatment with additional agents or radiation to the lungs, where residual disease remains. Choice B is incorrect as it only includes radiation to the flank and not the lungs, which are still harboring disease. Choice C is incorrect because although it adds cyclophosphamide and etoposide, it does not include radiation to the lungs, which is necessary for comprehensive disease control.
Question 2 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 3 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 4 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 5 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.