Of the variables listed below, what is the most important factor for survival after relapse of acute lymphoblastic leukemia?

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Question 1 of 5

Of the variables listed below, what is the most important factor for survival after relapse of acute lymphoblastic leukemia?

Correct Answer: A

Rationale: Step 1: Time to marrow relapse since initial diagnosis is crucial because early relapse indicates aggressive disease behavior. Step 2: Early relapse implies a higher likelihood of resistance to treatment, leading to poorer survival outcomes. Step 3: Rapid relapse often signifies a more challenging disease course, requiring prompt and aggressive interventions for better prognosis. Summary: - Choice A is correct as early relapse indicates aggressive disease behavior and resistance to treatment. - Choices B, C, and D are incorrect as they do not directly impact the urgency and aggressiveness of treatment needed for survival post-relapse.

Question 2 of 5

A 10-year-old girl has had transfusion-dependent anemia since age 6 months. She is found to have an unstable hemoglobin by sequence analysis (Hb Indianapolis). She has jaundice, obvious bony deformity from extramedullary hematopoiesis, and hepatosplenomegaly. Which of the following statements is correct?

Correct Answer: E

Rationale: Rationale for Correct Answer (E): The correct statement is likely about the fact that her hemoglobinopathy, Hb Indianapolis, is associated with ineffective erythropoiesis, leading to anemia, jaundice, and extramedullary hematopoiesis. This is a rare condition and not typically detected on newborn screens. Additionally, splenectomy may not entirely resolve her anemia due to the systemic nature of the disease. Since she has transfusion-dependent anemia, she is at risk for gallstones due to chronic hemolysis. Summary of Incorrect Choices: A: Incorrect because Hb Indianapolis is rare and not typically detected on newborn screens. B: Incorrect because extramedullary hematopoiesis can lead to nucleated red cells in the peripheral blood smear. C: Incorrect because the disease is systemic, and splenectomy may not fully resolve the anemia. D: Incorrect because chronic hemolysis from the disease increases the risk of gallstones despite transf

Question 3 of 5

A 15-year-old female presents with 1-month history of fatigue and a 3-day history of chest pain and shortness of breath. Her chest x-ray shows a large mediastinal mass that is greater than 33% of the thoracic diameter at the level of the diaphragm. A biopsy shows diffuse large B-cell lymphoma. Metastatic work-up, including a CT scan of neck, chest, abdomen, and pelvis; bone marrow biopsy; lumbar puncture; and PET scan show no other site of disease. According to the St. Jude (Murphy) staging system, what is the stage of this patient's non-Hodgkin lymphoma (NHL)?

Correct Answer: C

Rationale: The correct answer is C: Stage III. Rationale: 1. In the St. Jude (Murphy) staging system for non-Hodgkin lymphoma, Stage III is defined as the presence of a mediastinal mass that is greater than 33% of the thoracic diameter. 2. The patient in the scenario has a large mediastinal mass that meets this criteria, placing her in Stage III. 3. The absence of disease in other sites, as confirmed by the metastatic work-up, indicates that the disease is localized to the mediastinum. 4. Therefore, based on the specific criteria of the St. Jude (Murphy) staging system, the correct stage for this patient's non-Hodgkin lymphoma is Stage III. Summary of other choices: - Choice A: Stage I would not be appropriate as the mass is not localized to a single lymph node region. - Choice B: Stage II would not be correct as the mass extends beyond a

Question 4 of 5

A 9-year-old child with osteosarcoma is being admitted for cisplatin therapy. What is the best regimen for prevention of chemotherapy-induced nausea and vomiting (CINV)?

Correct Answer: C

Rationale: The correct answer is C: Granisetron, dexamethasone at 50% dosing, and арrepitant. This regimen is recommended by guidelines for moderate emetogenic chemotherapy in children. Granisetron is a first-line antiemetic for children, and combining it with dexamethasone and арrepitant provides a synergistic effect in preventing CINV. Dexamethasone at 50% dosing is preferred in children to reduce the risk of side effects. Palonosetron and olanzapine (choice A) are not typically recommended as first-line agents in pediatric patients. Dexamethasone and арrepitant (choice B) do not cover the full spectrum of CINV prevention compared to the correct regimen. Using dexamethasone at 100% dosing (choice D) may increase the risk of side effects in children without additional benefit in preventing CINV

Question 5 of 5

The patient is a 6-year-old boy referred to a hematologist for thrombocytopenia. He has no bleeding history or family history of bleeding. His only other past medical history is mild high-frequency hearing loss. What gene is responsible for these findings?

Correct Answer: C

Rationale: Rationale: The correct answer is C: MYH9, as mutations in the MYH9 gene are associated with May-Hegglin anomaly, which presents with thrombocytopenia and hearing loss. NBEAL2 (choice A) is linked to gray platelet syndrome, not hearing loss. GP-1Ba (choice B) is a platelet glycoprotein, not associated with hearing loss. Deletions of the long arm of chromosome 11 (choice D) are related to Jacobsen syndrome, which presents with thrombocytopenia but not hearing loss. Thus, based on the presented clinical findings, MYH9 is the most likely gene responsible.

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