ATI RN
ATI Hematologic System Questions
Question 1 of 5
A 15-year-old female presents with 1 month of fatigue and 3 days of chest pain and shortness of breath. Her physical exam is unremarkable. A chest x-ray shows a large mediastinal mass that is greater than 33% of the diameter of her chest cavity. A biopsy shows nodular sclerosing, classic Hodgkin lymphoma (cHL). Metastatic workup at diagnosis, including CT scan of neck, chest, abdomen, and pelvis and PET scan, shows no other site of disease. According to the Ann Arbor staging system, the patient has which stage of cHL?
Correct Answer: A
Rationale: The correct answer is A: Stage I. In the Ann Arbor staging system for Hodgkin lymphoma, Stage I indicates involvement of a single lymph node region (I) or a single extralymphatic site (IE) without systemic symptoms. In this case, the patient has a large mediastinal mass but no other sites of disease on metastatic workup. This corresponds to Stage I disease. Choice B: Stage II would indicate involvement of two or more lymph node regions on the same side of the diaphragm. Choice C: Stage III would indicate involvement of lymph node regions on both sides of the diaphragm. Choice D: Stage IV would indicate disseminated involvement of one or more extralymphatic organs or tissues. Therefore, based on the information provided, the correct stage for this patient is Stage I as per the Ann Arbor staging system.
Question 2 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 3 of 5
You are seeing a 2-year-old girl with new onset of fever and bronchitis. She has maculopapular rash and hepatosplenomegaly. Blood smear shows leukocytosis (100,000/mm3), anemia, and thrombocytopenia. Ancillary tests include fetal hemoglobin of 80% and normal blood karyotype. What is the most likely diagnosis?
Correct Answer: D
Rationale: The most likely diagnosis in this scenario is Juvenile myelomonocytic leukemia (JMML). This is supported by the presence of hepatosplenomegaly, maculopapular rash, leukocytosis, anemia, and thrombocytopenia in a young child. The elevated fetal hemoglobin level is characteristic of JMML. Additionally, a normal blood karyotype helps differentiate JMML from other leukemias. Choice A (Leukemoid Reaction) is incorrect because it is typically a reactive condition due to infections, not a primary hematological malignancy like JMML. Choice B (Acute lymphoblastic leukemia) is less likely due to the presence of hepatosplenomegaly and a high fetal hemoglobin level. Choice C (Chronic myeloid leukemia) is less likely in a young child with the given clinical presentation.
Question 4 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 5 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.