ATI RN
ATI Hematologic System Test Questions
Question 1 of 5
You are seeing a 12-year-old boy in the survivorship program who presented at 2 years old with a desmoplastic nodular medulloblastoma. You note the child recently underwent germline genetic testing and was found to have nevoid basal cell carcinoma syndrome. In which gene is the child most likely to have a pathogenic variant?
Correct Answer: C
Rationale: Rationale: 1. Nevoid basal cell carcinoma syndrome is also known as Gorlin syndrome, caused by mutations in SUFU. 2. SUFU gene regulates the Hedgehog signaling pathway, implicated in medulloblastoma development. 3. Desmoplastic nodular medulloblastoma is commonly seen in Gorlin syndrome. 4. PTEN is associated with Cowden syndrome, CDKN2A with melanoma, and SMARCB1 with rhabdoid tumors, not typically seen in Gorlin syndrome.
Question 2 of 5
A 14-year-old boy presents with cough, shortness of breath, and difficulty lying down. His face and neck swell when his arms are raised. Chest x-ray reveals a large mediastinal mass. A tissue diagnosis is desired. A biopsy is performed with local anesthesia because the anesthesiologist thinks that the patient has a very high general anesthesia risk. Which of the following findings does not make general anesthesia unsafe?
Correct Answer: D
Rationale: The correct answer is D. A malignancy of hematopoietic origin does not inherently make general anesthesia unsafe. This is because anesthesia risk is typically influenced by factors such as airway patency, respiratory function, and cardiac status. Choices A, B, and C all indicate potential complications that could make general anesthesia unsafe, such as airway obstruction due to a large mediastinal mass (A), compromised tracheal cross-sectional area (B), and impaired respiratory function (C). These factors would necessitate careful consideration and potential modifications in the anesthesia plan to ensure patient safety.
Question 3 of 5
A leukemia investigator plans to obtain bone marrow under general anesthesia to measure minimal residual disease (MRD) and to see if this time point can predict early relapse. The specimen will be obtained at a time point when otherwise no bone marrow would be sampled. The results are not shared with the treating oncologist, and no therapeutic interventions are decided or based on the results. Which of the following statements is most accurate about this intervention?
Correct Answer: C
Rationale: The correct answer is C because obtaining bone marrow under general anesthesia carries inherent risks that make it greater than minimal-risk. Here's a step-by-step rationale: 1. General anesthesia poses risks such as allergic reactions, breathing problems, and potential complications, making the procedure more than minimal-risk. 2. Even though bone marrow assessments are routine for leukemia patients, the use of general anesthesia elevates the risk level. 3. The fact that the results are not shared with the treating oncologist and no therapeutic interventions are based on them indicates that the procedure is solely for research purposes, emphasizing the risk-benefit analysis. 4. Choice A is incorrect because the use of general anesthesia increases the procedure's risk level beyond routine. 5. Choice B is incorrect as the focus should be on the inherent risks of general anesthesia, not just the number of procedures performed. 6. Choice D is irrelevant to the risk assessment of the specific procedure and does not address the immediate safety concerns associated with general anesthesia.
Question 4 of 5
A 7-year-old boy presents with recent onset of vomiting and lethargy. Blood smear shows increased neutrophils with a left shift and 8% abnormal cells. Bone marrow contains 60% of the same cells. Flow cytometry shows that the cells are TdT–, CD10+, CD19+, CD20+, sIg+. What is the most likely diagnosis?
Correct Answer: A
Rationale: The correct answer is A: Burkitt leukemia/lymphoma. This diagnosis is supported by the presence of abnormal cells that are CD10+, CD19+, CD20+, sIg+, which are characteristic of Burkitt lymphoma. The absence of TdT– indicates that it is not T-cell ALL. B-cell ALL would typically show TdT positivity. Hematogones are immature B-cell precursors commonly seen in the bone marrow of healthy children and do not typically present with the described clinical picture. The high percentage of abnormal cells in both blood and bone marrow, along with the specific immunophenotype, point towards Burkitt leukemia/lymphoma.
Question 5 of 5
An infant is born with a firm mass over the chest with a central area of purpura and a 'halo' around it. An ultrasound reveals a high-flow lesion. What is the most likely diagnosis?
Correct Answer: C
Rationale: The most likely diagnosis in this case is congenital hemangioma (Choice C). This is because the description of a firm mass over the chest with central purpura and a 'halo' suggests a rapidly involuting congenital hemangioma. The high-flow lesion seen on ultrasound is characteristic of this type of hemangioma. Choice A (Fibrosarcoma) is incorrect as fibrosarcoma typically presents differently and does not typically have a central area of purpura. Choice B (Infantile hemangioma) is incorrect as infantile hemangiomas are not usually present at birth and do not typically have a 'halo' appearance. Choice D (Capillary malformation) is incorrect as capillary malformations do not usually present as a firm mass with central purpura and a 'halo'. In summary, the key features described in the question point towards a diagnosis of congenital hemangioma, making Choice C the most likely