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: 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 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 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 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 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.

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 family history, particularly the sister's unresponsiveness to ATG and cyclosporine and the cousin's history of leukemia, raises suspicion for Fanconi anemia. Testing for Fanconi anemia is crucial as it is an inherited bone marrow failure syndrome that predisposes individuals to aplastic anemia and leukemia. Skin fibroblast culture is the preferred test for diagnosing Fanconi anemia as it can detect chromosomal abnormalities indicative of the disease. Options A and B are not appropriate as the patient's sister did not respond to ATG and cyclosporine, and searching for a donor for transplant without confirming the underlying cause of aplastic anemia could lead to transplant failure. Option C is also not the best choice as the peripheral blood sample test for Fanconi anemia was already negative, and a skin fib

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: The correct answer is B because reduced intensity regimens are used to minimize toxicity and late effects, not for most nonmalignant disorders. Myeloablative approaches are needed for high-risk malignancies to maximize remission depth (A), reduced intensity regimens can increase transplant-related mortality in high-risk patients (C), and non-myeloablative regimens are used for the highest risk patients and certain diseases like aplastic anemia (D).

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