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: The correct answer is C: SUFU. Nevoid basal cell carcinoma syndrome, also known as Gorlin syndrome, is associated with pathogenic variants in the SUFU gene. SUFU is a tumor suppressor gene involved in the sonic hedgehog signaling pathway, which plays a role in medulloblastoma and basal cell carcinoma development. PTEN (A) is associated with Cowden syndrome, CDKN2A (B) with familial atypical multiple mole melanoma syndrome, and SMARCB1 (D) with rhabdoid tumor predisposition syndrome, but not specifically linked to nevoid basal cell carcinoma 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 because a malignancy of hematopoietic origin does not inherently increase the risk of general anesthesia. Hematopoietic malignancies such as leukemia or lymphoma do not directly impact the respiratory system or airway, unlike the other choices. A: Tumor size affecting transthoracic diameter can compress airways, leading to respiratory compromise. B: Tracheal cross-sectional area affects air flow and can be a concern during intubation. C: Peak expiratory flow rate indicates respiratory function; lower values suggest increased risk under general anesthesia. Therefore, D is the correct answer as it does not pose a direct risk to general anesthesia.
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: Step 1: General anesthesia carries inherent risks, making the procedure more than minimal-risk. Step 2: The absence of therapeutic interventions based on results does not make the procedure low-risk. Step 3: Performing an additional procedure does not automatically make it minimal-risk. Step 4: Not sharing results with the treating oncologist may raise ethical concerns. Summary: Choice C is correct because the use of general anesthesia increases the risk level of the procedure, making it greater than minimal-risk. Choices A, B, and D are incorrect as they do not adequately address the specific risk associated with the use of general anesthesia in this scenario.
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. The key features in the case are increased neutrophils with a left shift, presence of abnormal cells in blood and bone marrow, and flow cytometry showing TdT–, CD10+, CD19+, CD20+, sIg+. These findings are consistent with Burkitt leukemia/lymphoma, a highly aggressive B-cell neoplasm. The presence of CD10, CD19, and CD20, along with sIg positivity, points towards a B-cell origin. Additionally, the characteristic translocation t(8;14) involving c-Myc oncogene is commonly seen in Burkitt lymphoma. The absence of TdT expression rules out T-cell ALL (Choice C). Hematogones (Choice D) are normal precursor B-cells found in bone marrow, but they do not typically present with the clinical features described. B-cell ALL (Choice B) lacks the specific features seen in this case
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 correct answer is C: Congenital hemangioma. A congenital hemangioma presents as a firm mass with purpura and a 'halo' due to rapid growth of blood vessels. The central area of purpura indicates thrombosis. The high-flow lesion on ultrasound suggests an increased blood flow. Fibrosarcoma (A) is a malignant tumor of fibrous tissue and does not present with these characteristic features. Infantile hemangioma (B) typically appears after birth and has a different clinical presentation. Capillary malformation (D) is a vascular malformation that does not typically show the same features as described in the question.