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

Correct Answer: A

Rationale: The correct answer is A: Time to marrow relapse since initial diagnosis. This is because the time to marrow relapse indicates the aggressiveness of the leukemia and how quickly it has returned post-treatment. Quicker relapse indicates more aggressive disease, impacting survival. Choice B, sex, is not a significant factor in survival post-relapse. Choice C, central nervous system involvement, affects treatment but is not the primary factor for survival. Choice D, response to induction therapy, is crucial for initial treatment success but not the most important factor post-relapse survival. Therefore, choice A is the most critical factor for survival after relapse of acute lymphoblastic leukemia.

Correct Answer: E

Rationale: Step 1: Hb Indianapolis is a rare unstable hemoglobin variant causing severe hemolytic anemia. Step 2: The clinical presentation includes jaundice, bony deformity, hepatosplenomegaly due to extramedullary hematopoiesis. Step 3: None of the choices directly address the specific characteristics of Hb Indianapolis. Step 4: Therefore, the correct answer must provide insight into the unique features of this condition. Step 5: Choice E is correct as it highlights that the clinical presentation of Hb Indianapolis is distinct from other beta-hemoglobinopathies. Step 6: Summary: Choices A, B, C, and D are incorrect as they do not address the specific features of Hb Indianapolis, unlike choice E.

Correct Answer: C

Rationale: The correct answer is C: Stage III. According to the St. Jude (Murphy) staging system for non-Hodgkin lymphoma, Stage III is defined as disease that extends beyond a single region (e.g., thorax) on one side of the diaphragm. In this case, the large mediastinal mass greater than 33% of thoracic diameter indicates involvement beyond a single region. The absence of disease in other sites based on the metastatic work-up rules out Stages IV. Since there is no information provided to suggest involvement on both sides of the diaphragm, Stage III is the most appropriate stage for this patient. Choice A (Stage I) is incorrect as the disease extends beyond a single region. Choice B (Stage II) is incorrect as it implies involvement on both sides of the diaphragm, which is not indicated. Choice D (Stage IV) is incorrect as there is no evidence of distant metastasis in the given scenario.

Correct Answer: C

Rationale: The correct answer is C: Granisetron, dexamethasone at 50% dosing, and aprepitant. Granisetron is a first-line antiemetic for CINV in chemotherapy. Dexamethasone at 50% dosing is effective in reducing nausea and vomiting. Aprepitant, a neurokinin-1 receptor antagonist, is recommended for moderate to high emetogenic chemotherapy regimens like cisplatin. This combination provides a comprehensive approach targeting different pathways involved in CINV. Choice A is incorrect because olanzapine is not typically used in pediatric patients for CINV prevention. Choice B is incorrect as aprepitant is preferred over olanzapine. Choice D is incorrect because excessive dexamethasone dosing can increase the risk of side effects without additional benefit.

Correct Answer: C

Rationale: Rationale for correct answer (C - MYH9): 1. MYH9 gene encodes for non-muscle myosin heavy chain IIA. 2. Mutations in MYH9 gene are associated with May-Hegglin anomaly, characterized by thrombocytopenia without bleeding tendency. 3. Additionally, MYH9-related disorders can present with sensorineural hearing loss. 4. Given the patient's thrombocytopenia and hearing loss, MYH9 gene is the most likely culprit. Summary of incorrect choices: A: NBEAL2 - Associated with gray platelet syndrome, not consistent with patient's presentation. B: GP-1Ba - Glycoprotein Ib-IX-V complex gene, not linked to the symptoms described. D: Deletions of long arm of chromosome 11 - Not specific to thrombocytopenia and hearing loss in this context.