ATI RN
Anatomy of Hematologic System Questions
Question 1 of 5
A 5-year-old boy presents with a 3-week history of his right eye “not moving to the right side.†The remainder of his neurologic exam is normal with the exception of a weak gag. MRI of the brain reveals a diffusely infiltrative mass in the pons. If this patient were to undergo a stereotactic biopsy, what would be the most likely molecular finding?
Correct Answer: C
Rationale: The correct answer is C: H3 K27M mutation. In children with diffuse midline gliomas, the most common molecular finding is the H3 K27M mutation. This mutation is associated with a worse prognosis and is commonly found in tumors located in the pons, which is consistent with the MRI findings in this case. Choice A (BRAFKIAA1549 fusion) is incorrect as this fusion is typically associated with pilocytic astrocytomas, which are low-grade tumors and not typically found in the pons. Choice B (Trisomy 21) is incorrect as it is a chromosomal abnormality associated with Down syndrome, not a molecular finding in brain tumors. Choice D (RELA fusion) is incorrect as this fusion is typically found in ependymomas, which are not typically located in the pons.
Question 2 of 5
A 10-year-old girl is a long-term survivor of type II pleuropulmonary blastoma (PPB). You suspect she has a cancer predisposition syndrome and perform genetic testing, which confirms she has DICER1 syndrome. Which other cancer is she predisposed to?
Correct Answer: A
Rationale: The correct answer is A: Papillary thyroid cancer. DICER1 syndrome is associated with an increased risk of developing various tumors, including papillary thyroid cancer. This is due to the role of DICER1 gene mutation in dysregulating cell growth and division. Other choices like B: Medullary thyroid cancer, C: Pheochromocytoma, and D: Renal cell carcinoma are not typically associated with DICER1 syndrome. Medullary thyroid cancer is more commonly linked to RET gene mutations, pheochromocytoma to RET or SDHB/C/D gene mutations, and renal cell carcinoma to VHL gene mutations. Therefore, based on the genetic mechanism and known associations, papillary thyroid cancer is the most likely cancer this patient is predisposed to.
Question 3 of 5
A 17-year-old patient is referred to you for a platelet count of 1,200,000/mm3. On history, she notes that she often has numbness and tingling in her hands and feet and has frequent epistaxis. She is otherwise well-appearing and has no recent infections. On her exam, you note splenomegaly. What do you expect to see on further evaluation?
Correct Answer: D
Rationale: The correct answer is D: Low von Willebrand factor activity. This patient likely has Essential Thrombocythemia, a disorder characterized by excessive production of platelets by the bone marrow. This condition can lead to abnormal bleeding and clotting. The patient's symptoms of numbness, tingling, epistaxis, and splenomegaly are consistent with this diagnosis. Low von Willebrand factor activity is expected in Essential Thrombocythemia due to platelet dysfunction. Elevated C-reactive protein, low ferritin, and a hypocellular bone marrow are not typically associated with Essential Thrombocythemia and are less likely to be seen in this patient.
Question 4 of 5
A 13-year-old boy presents to the emergency department with complaints of headache and visual changes. History reveals progressive dyspnea on exertion, generalized fatigue, and increased bruising. His labs are significant for a WBC of 350,000/mcL, of which 80% are reported to be blasts and appear to be myeloblasts without the presence of Auer rods. His hemoglobin is 7.2 g/dL, and his platelets are 18,000/mcL. A CT scan of the head shows a small intracerebral hemorrhage. His coags are normal. Which of the following is the most appropriate therapy?
Correct Answer: C
Rationale: The most appropriate therapy in this scenario is emergent leukapheresis plus hydroxyurea (Choice C). Leukapheresis is needed to rapidly reduce the high white blood cell count, preventing further complications such as leukostasis and hyperviscosity syndrome. Hydroxyurea can help further control the rapid proliferation of blasts. Induction chemotherapy (Choice A) may lead to tumor lysis syndrome due to the high tumor burden. Performing leukapheresis alone without a subsequent therapy (Choice B) may not adequately address the underlying disease. Providing cranial radiation (Choice D) is not the initial management for acute myeloid leukemia.
Question 5 of 5
What are the six dimensions of quality care according to the Institute of Medicine?
Correct Answer: A
Rationale: The correct answer is A: Safe, timely, effective, efficient, equitable, and person-centered. According to the Institute of Medicine, these six dimensions encompass quality care. Safety is essential to avoid harm. Timeliness ensures care is provided promptly. Effectiveness means care should be evidence-based and achieve desired outcomes. Efficiency aims to minimize waste. Equitability ensures fair and non-discriminatory care. Lastly, being person-centered emphasizes individual preferences and needs. Summary: Choice B is incorrect because transparency is not included in the six dimensions. Choice C is incorrect as low-cost is not a primary dimension of quality care. Choice D is incorrect as it includes cutting-edge, which is not one of the six dimensions outlined by the Institute of Medicine.