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 likely has hereditary elliptocytosis, an autosomal dominant disorder caused by mutations in the alpha-spectrin gene. Ovalocytes and polkilocytosis are characteristic of this condition. Since both parents have ovalocytes, it is likely that the infant inherited mutations from each parent, resulting in a more severe presentation. Anemia in hereditary elliptocytosis can improve over time due to compensatory mechanisms. Choices A, B, and D are incorrect as they describe different genetic disorders (hereditary spherocytosis, hereditary stomatocytosis, and pyruvate kinase deficiency, respectively) and do not match the clinical and family history provided.
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: The correct answer is D because the patient with acute lymphoblastic leukemia and symptoms of lethargy, flank pain, nausea, and vomiting is likely experiencing tumor lysis syndrome. This syndrome can lead to hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalcemia, and elevated BUN levels. In option D, the potassium, phosphorus, and BUN levels are elevated, while the calcium level is decreased, which aligns with the expected findings in tumor lysis syndrome. The other choices do not reflect the characteristic electrolyte imbalances seen in tumor lysis syndrome.
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 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 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 case is Juvenile myelomonocytic leukemia (JMML). JMML is a rare myelodysplastic/myeloproliferative neoplasm seen in young children. The clinical presentation of fever, rash, hepatosplenomegaly, leukocytosis, anemia, and thrombocytopenia is consistent with JMML. The presence of fetal hemoglobin of 80% is a key finding in JMML, as it is a distinguishing feature. Additionally, a normal blood karyotype rules out chromosomal abnormalities commonly seen in other leukemias. Leukemoid reaction (Choice A) is characterized by a reactive increase in leukocyte count due to an underlying condition, but it does not explain the other findings in this case. Acute lymphoblastic leukemia (ALL - Choice B) primarily affects lymphoid cells, not myeloid cells as seen in this case. Chronic myeloid leukemia (C
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 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.