ATI RN
ATI Hematologic System Questions
Question 1 of 5
A newborn infant develops jaundice on day of life 2. Labs are drawn, and she has a hemoglobin of 7.4 g/dL with a reticulocyte count of 8%. Upon peripheral blood smear review, she is found to have bizarre red cell forms with significant polkilocytosis. Although her parents have normal blood counts, on review of their peripheral blood smears, they both have a moderate number of ovalocytes. Which of the following is the most likely cause of the infant's red cell findings?
Correct Answer: C
Rationale: The correct answer is C because the infant's red cell findings of ovalocytes and polikilocytosis are consistent with hereditary elliptocytosis, which is caused by mutations in genes encoding spectrin. In this scenario, the inheritance pattern aligns with the infant receiving an alpha-spectrin mutation from both parents, leading to the observed erythrocyte abnormalities. This condition may improve over time due to compensatory mechanisms. Choice A (autosomal dominant ankyrin mutation causing hereditary spherocytosis) is incorrect because the clinical presentation and red cell morphology do not support a diagnosis of hereditary spherocytosis. Choice B (inherited band 3 variants and need for splenectomy) is incorrect as the infant's condition does not match the characteristics of hereditary spherocytosis requiring splenectomy. Choice D (PKLR variant and pyruvate kinase deficiency) is incorrect as the infant's red cell morphology is not indicative
Question 2 of 5
When reviewing the chemistry panel of a newly diagnosed patient with acute lymphoblastic leukemia who is lethargic, complaining of flank pain, and experiencing nausea and vomiting, which of the following would you expect to see?
Correct Answer: D
Rationale: Step-by-step rationale: 1. Lethargy, flank pain, nausea, vomiting in leukemia can suggest tumor lysis syndrome (TLS). 2. TLS can cause hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalcemia, and elevated BUN. 3. Choice D has the highest potassium, phosphorus, uric acid, and BUN levels, and the lowest calcium level. 4. Therefore, choice D is the most consistent with the expected lab findings in tumor lysis syndrome. Summary: - Choice A has normal potassium, phosphorus, uric acid, calcium levels, and lower BUN. - Choice B has high potassium but normal phosphorus, uric acid, calcium, and slightly elevated BUN. - Choice C has low potassium, normal phosphorus, and elevated uric acid, calcium, and BUN. - Choice D aligns most closely with the expected lab findings in tumor lysis syndrome due to the pattern of
Question 3 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 4 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 5 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