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 relevant consideration in determining whether a project is research or quality improvement. Here's a step-by-step rationale: 1. Quality improvement aims to enhance processes within a specific institution, while research seeks to generate generalizable knowledge. 2. Methodology using Plan-Do-Study-Act cycles is common in both quality improvement and research projects. 3. Efforts to stabilize biases/confounders over time align with both quality improvement and research principles. 4. Intent to publish in a peer-reviewed journal does not define the project as research; it is possible to publish quality improvement initiatives as well.
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: Begin therapy with all-trans retinoic acid (ATRA) immediately while aggressively managing coagulopathy with blood product support. In this scenario, the patient presents with acute promyelocytic leukemia (APL), characterized by the presence of Auer rod-containing blasts expressing CD33 and negative for HLA-DR. The presence of coagulopathy with elevated INR, D-dimer, and oozing blood suggests disseminated intravascular coagulation (DIC), a common complication in APL. Immediate treatment with ATRA is crucial to differentiate and mitigate the risk of DIC worsening. ATRA induces differentiation of APL blasts, resolving the coagulopathy. Aggressive management of coagulopathy with blood product support is essential to prevent bleeding complications. Lumbar puncture (choice A), dexamethasone and hydroxyurea (choice C), and starting a donor search (choice D) are not indicated as the
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 history, including a family member with aplastic anemia and a cousin with leukemia, raises suspicion for a genetic disorder like Fanconi anemia. Testing skin fibroblasts for Fanconi anemia can help confirm or rule out this diagnosis. Administering ATG and cyclosporine (choice A) may not be effective if the underlying cause is a genetic disorder. Searching for a donor for matched unrelated transplant (choice B) is premature without confirming the diagnosis. Sending a bone marrow aspirate for Fanconi anemia testing (choice C) may not yield accurate results as the peripheral blood sample test was negative, making skin fibroblast culture the preferred choice.
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: B is the correct answer because reduced intensity regimens are not suitable for most nonmalignant disorders. Myeloablative approaches are typically used for high-risk malignancies to maximize remission depth and reduce relapse likelihood. Reduced intensity regimens are used for patients with significant comorbidities to decrease transplant-related mortality, but may lead to more relapse and graft-versus-host disease. Non-myeloablative regimens are utilized for high-risk patients to minimize toxicity and for specific diseases like aplastic anemia.
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: The correct answer is C: Interleukin-1 receptor-associated kinase 4 (IRAK4). 1. IRAK4 is involved in the immune response pathway triggered by interleukin-1 receptor signaling. 2. Lack of Toll-like receptor responses in the patient aligns with the role of IRAK4 in the interleukin-1 receptor pathway. 3. Pathogenic variants in IRAK4 can lead to immunodeficiency, explaining recurrent Staph infections. 4. Choices A, B, and D do not accurately reflect the known function of IRAK4 and its association with interleukin-1 receptor signaling.