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 addition of cyclophosphamide and etoposide to the chemotherapy regimen is indicated for patients with combined loss of heterozygosity for 1p and 16q in Wilms' tumor. This genetic abnormality is associated with a higher risk of relapse and poorer outcomes. The extended duration of chemotherapy (33 weeks) is necessary to target any remaining tumor cells and reduce the risk of recurrence. Additionally, the decision to administer radiation to both the lungs and flank is appropriate given the incomplete resolution of some lung nodules after initial chemotherapy. Lung radiation helps to target any remaining metastatic lesions, while flank radiation targets the primary tumor site and positive lymph nodes. This comprehensive treatment approach aims to maximize the chances of long-term disease control and survival for the patient. Choice A is incorrect because it lacks the addition of cyclophosphamide and etoposide, which are needed for Wilms' tumor with the specific genetic abnormality mentioned.
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 case, the 4-year-old girl with a factor VIII deficiency did not respond to recombinant factor VIII, suggesting a possible von Willebrand disease (vWD) as well. Infusing von Willebrand factor concentrate can help address the underlying vWD component, which is necessary for adequate hemostasis. Option B is incorrect because giving another dose of recombinant factor VIII won't address the potential vWD deficiency. Option C is not the best next step as packing the nose does not address the underlying bleeding disorder. Option D is not the immediate next step as checking for a factor VIII inhibitor is important but can be done after addressing the acute bleeding episode with appropriate therapy.
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, which are a major source of HLA antigens that can trigger alloimmunization. By using leukoreduced blood products, the risk of developing anti-HLA antibodies is significantly reduced, thus minimizing the potential for alloimmunization prior to transplant. A: Frozen RBCs - Freezing does not eliminate HLA antigens and does not prevent alloimmunization. B: Volume-reduced blood products - Reducing volume does not impact HLA antigens and thus doesn't prevent alloimmunization. C: Irradiation of all blood products - While irradiation prevents graft-versus-host disease, it does not directly prevent the development of anti-HLA antibodies.
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 indicates iron deficiency anemia. The low Hgb of 8.7 g/dL is below normal range for his age. The MCV of 60 fL is low, indicating microcytic anemia which is characteristic of iron deficiency. The serum ferritin level of 2 ng/mL is very low, supporting the diagnosis. Choice B is incorrect as the Hgb and MCV are within normal range, and the ferritin level is not indicative of iron deficiency. Choice C has a low Hgb but normal MCV and ferritin level. Choice D has a normal Hgb and low ferritin, but the MCV is high, inconsistent with iron deficiency anemia.
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. Given the complete resection with clean margins, normal chest CT, bone scan, and absence of NF1, observation is appropriate. Chemotherapy or radiotherapy may be considered in the presence of residual disease, positive margins, or metastasis. Chemotherapy with doxorubicin and ifosfamide may cause unnecessary toxicity without clear benefit in this scenario. Radiotherapy may be reserved for cases with high-risk features. Combining chemotherapy and radiotherapy may lead to increased toxicity without proven benefit. Observation allows monitoring for disease recurrence while minimizing unnecessary treatment side effects.