ATI RN
Anatomy of Hematologic System Questions
Question 1 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 2 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 3 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.
Question 4 of 5
You are seeing a 13-year-old boy with fatigue, weight loss, night sweats, and splenomegaly. Peripheral blood shows anemia, thrombocytosis, and leukocytosis (300,000/mm3). What is this patient's most likely diagnosis?
Correct Answer: C
Rationale: The most likely diagnosis for this 13-year-old boy presenting with fatigue, weight loss, night sweats, splenomegaly, anemia, thrombocytosis, leukocytosis (300,000/mm3) is Chronic Myeloid Leukemia (CML). 1. Presence of anemia, thrombocytosis, and leukocytosis is characteristic of CML. 2. CML typically presents with splenomegaly. 3. Age of onset (13 years) is within the typical range for CML. 4. The Philadelphia chromosome (t(9;22)) is present in most cases of CML, aiding in diagnosis. 5. Leukemoid reaction (A) is a reactive increase in white blood cells, not a primary neoplastic disorder. 6. Acute lymphoblastic leukemia (ALL) (B) typically presents with bone pain, lymphadenopathy, and CNS symptoms in children. 7
Question 5 of 5
An infant is born with a 7 cm × 6 cm lesion over the upper extremity from the elbow to the shoulder. The lesion is indurated and purpuric, with some petechiae around the edges. No other areas of petechiae are noted on the skin. The infant is doing well without other systemic problems. i were 9 and 9. You are called by the pediatric nurse practitioner to the NICU. What is the most appropriate next step?
Correct Answer: D
Rationale: The correct answer is D: Obtain labs, including a CBC with platelet count and fibrinogen. This is the most appropriate next step because the infant's presentation with an indurated and purpuric lesion raises concern for a possible bleeding disorder or coagulopathy. By obtaining labs, including a CBC with platelet count and fibrinogen, we can assess the infant's hemostatic profile and rule out any underlying hematologic abnormalities. This step is crucial in ensuring the infant's well-being and guiding further management if any abnormalities are detected. Why the other choices are incorrect: A: Doing nothing is not appropriate as the presentation warrants further investigation. B: An ultrasound may not provide useful information in this scenario as the lesion is concerning for a systemic issue. C: An MRI is not the initial diagnostic test of choice for evaluating a possible bleeding disorder or coagulopathy.