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. This mutation is most commonly associated with diffuse intrinsic pontine gliomas (DIPG), which are high-grade brain tumors typically found in children. The presence of a diffusely infiltrative mass in the pons in a 5-year-old boy with neurological symptoms points towards a DIPG. Stereotactic biopsy of the mass would likely reveal the H3 K27M mutation, which is a key molecular finding in DIPG. Choice A (BRAFKIAA1549 fusion) is incorrect as this fusion is typically associated with pilocytic astrocytomas, not DIPGs. Choice B (Trisomy 21) is incorrect as it is not a molecular finding commonly associated with DIPGs. Choice D (RELA fusion) is also incorrect as this fusion is more commonly seen in ependymomas, not DIPGs.
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. Individuals with DICER1 syndrome are at increased risk for developing various tumors, including papillary thyroid cancer. This is due to the role of DICER1 gene in regulating cell growth and division. The other choices, B (medullary thyroid cancer), C (pheochromocytoma), and D (renal cell carcinoma), are not typically associated with DICER1 syndrome. Medullary thyroid cancer is commonly linked to RET gene mutations, pheochromocytoma to genes such as SDHB, SDHD, and VHL, and renal cell carcinoma to mutations in genes like VHL and MET.
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. A platelet count of 1,200,000/mm3 suggests thrombocytosis, which can be seen in conditions like essential thrombocythemia. This condition is associated with low von Willebrand factor activity, leading to increased bleeding tendency despite high platelet counts. Numbness, tingling, epistaxis, and splenomegaly are common features of essential thrombocythemia. Elevated C-reactive protein is not typically associated with this condition. Low ferritin is more indicative of iron deficiency anemia, not thrombocytosis. A hypocellular bone marrow is not expected in essential thrombocythemia, as it is characterized by increased platelet production.
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 correct answer is C: Perform emergent leukapheresis plus hydroxyurea. In this scenario, the patient presents with symptoms suggestive of acute myeloid leukemia with hyperleukocytosis and intracerebral hemorrhage. The goal of emergent therapy is to rapidly reduce the high blast count to prevent further complications such as leukostasis and hemorrhage. Leukapheresis can provide immediate reduction in the blast count, while hydroxyurea can further decrease the WBC count. Starting induction chemotherapy immediately can lead to tumor lysis syndrome due to rapid cell destruction. Emergent cranial radiation is not the primary intervention for hyperleukocytosis. Performing leukapheresis alone without adjunctive therapy like hydroxyurea may not adequately control the blast count. Thus, the most appropriate initial therapy is emergent leukapheresis plus hydroxyurea to stabilize the patient before initiating induction chemotherapy.
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. This aligns with the six dimensions of quality care outlined by the Institute of Medicine. Safety ensures patient well-being, timeliness reduces delays, effectiveness means providing evidence-based care, efficiency minimizes waste, equity ensures fair access, and person-centered care considers individual preferences. Choice B is incorrect because transparency is not included in the Institute of Medicine's six dimensions. Choice C is incorrect as "low-cost" is not a recognized dimension of quality care. Choice D is incorrect because "cutting-edge" is not part of the established dimensions of quality care.