ATI RN
ATI Hematologic System Questions
Question 1 of 5
A pediatric fellow is planning a project intended to decrease the incidence of acute chest syndrome among patients with sickle cell disease who are already admitted to the hospital for other reasons. The fellow discussed with her mentor whether the project proposal should be submitted for review by the Institutional Review Board (IRB). The mentor explains that, at their intuition, quality improvement activities do not require IRB review but research projects must be submitted to the IRB. Which of the following is NOT a relevant consideration in determining whether the project is research or quality improvement?
Correct Answer: C
Rationale: The correct answer is C because the intent to publish results in a peer-reviewed journal is not a determining factor in distinguishing between quality improvement and research activities. Here's a step-by-step rationale: 1. Quality improvement focuses on improving processes within an institution to enhance patient care, while research aims to generate new knowledge that is generalizable beyond the institution. 2. The chosen methodology (B) such as using Plan-Do-Study-Act cycles is often associated with quality improvement projects to test and implement changes iteratively. 3. Efforts to hold biases stable over time (D) are more aligned with research, as quality improvement projects may not necessarily require controlling for biases like randomization. 4. The intent to publish in a peer-reviewed journal (C) can apply to both quality improvement and research projects, but the key factor in determining the need for IRB review is whether the project aims to generate generalizable knowledge (research) or improve local processes (quality improvement).
Question 2 of 5
A 13-year-old Hispanic girl is found to have a WBC count of 6,500/mm3 with 40% Auer rod–containing granular blasts that, by flow cytometry, express very bright CD33 but are negative for human leukocyte antigen–DR isotype (HLA-DR). She is oozing blood around her peripheral IV site. Coagulation studies reveal an international normalized ratio (INR) of 3.4, a fibrinogen of 170, and a markedly elevated D-dimer. Marrow aspirate shows nearly 90% blasts with a similar morphology. You send the marrow to the fluorescence in situ hybridization (FISH) lab and request STAT testing for the most likely recurrent genetic abnormality based on the clinical presentation. How do you plan to initiate therapy?
Correct Answer: B
Rationale: The correct answer is B because the clinical presentation suggests acute promyelocytic leukemia (APL). APL is characterized by Auer rod-containing granular blasts, which are positive for CD33 and negative for HLA-DR. The presence of coagulopathy with elevated D-dimer and oozing blood indicates a high risk of disseminated intravascular coagulation (DIC), a common complication of APL. Immediate treatment with all-trans retinoic acid (ATRA) is crucial in APL to induce differentiation of the leukemic cells and prevent further coagulopathy. Aggressively managing the coagulopathy with blood product support is also important to stabilize the patient. Choice A is incorrect because a lumbar puncture is not necessary for APL diagnosis, and immediate induction chemotherapy with ATRA is the standard of care. Choice C is incorrect because dexamethasone and hydroxyurea are not the first-line therapies for APL.
Question 3 of 5
You examine a 10-year-old boy with severe aplastic anemia. He has no dysmorphic features and is at the 50th percentile for height and weight. Family history includes a sister with aplastic anemia unresponsive to anti-human thymocyte globulin (ATG) and cyclosporine who died early in the course of an unrelated donor hematopoietic stem cell transplant complicated by severe mucositis and transplant-related organ toxicities. There are no other siblings. A cousin died of acute myeloid leukemia at age 5 years. A peripheral blood sample test for Fanconi anemia is negative with no increased chromosomal breaks in response to diepoxylbutane or mitomycin C. Which of the following is the most important next step in management?
Correct Answer: D
Rationale: The correct answer is D: Send a skin fibroblast culture for Fanconi anemia testing. This is the most important next step in management because the patient's family history, particularly the sister's unresponsiveness to ATG and cyclosporine and the cousin's history of leukemia, raises suspicion for Fanconi anemia. Testing for Fanconi anemia is crucial as it is an inherited bone marrow failure syndrome that predisposes individuals to aplastic anemia and leukemia. Skin fibroblast culture is the preferred test for diagnosing Fanconi anemia as it can detect chromosomal abnormalities indicative of the disease. Options A and B are not appropriate as the patient's sister did not respond to ATG and cyclosporine, and searching for a donor for transplant without confirming the underlying cause of aplastic anemia could lead to transplant failure. Option C is also not the best choice as the peripheral blood sample test for Fanconi anemia was already negative, and a skin fib
Question 4 of 5
Which of the following statements about myeloablative, myeloablative but reduced toxicity, reduced intensity, and non-myeloablative approaches is not correct?
Correct Answer: B
Rationale: The correct answer is B because reduced intensity regimens are used to minimize toxicity and late effects, not for most nonmalignant disorders. Myeloablative approaches are needed for high-risk malignancies to maximize remission depth (A), reduced intensity regimens can increase transplant-related mortality in high-risk patients (C), and non-myeloablative regimens are used for the highest risk patients and certain diseases like aplastic anemia (D).
Question 5 of 5
A 4-year-old male child presents to the emergency department with his fourth invasive Staph infection. CBC consistently identifies moderate neutropenia. Sophisticated lab testing identifies lack of Toll-like receptor responses. The patient undergoes whole exome sequencing and is found to have pathogenic variants in IRAK4. What does 'IRAK4' stand for?
Correct Answer: C
Rationale: Step 1: Identify the function of IRAK4. IRAK4 is involved in the signaling pathway of the immune system, particularly in response to interleukin-1 (IL-1) receptor activation. Step 2: Break down the acronym IRAK4. IRAK4 stands for Interleukin-1 Receptor-Associated Kinase 4. Step 3: Link the information in the question to the correct answer. Given that the patient has a lack of Toll-like receptor responses and pathogenic variants in IRAK4, it indicates a problem with the interleukin-1 signaling pathway, making choice C (Interleukin-1 receptor-associated kinase 4) the correct answer. Summary: A: Incorrect - Interferon gamma receptor-associated kinase does not match the function of IRAK4. B: Incorrect - Inducible RAS activating kinase does not match the function of IRAK4. C: Correct - Matches the function of IRAK4