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?

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Question 1 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 2 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 3 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 4 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 5 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.

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