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.

Correct Answer: C

Rationale: The correct answer is C: Antifungal prophylaxis. This patient is at high risk for fungal infections post-bone marrow transplantation due to the conditioning regimen with cyclophosphamide and total body irradiation. CMV and HSV prophylaxis are not indicated as the patient and donor are negative for CMV IgG and IgM and the donor is positive for HSV antibodies. Weekly CMV PCR screening is not necessary in the absence of CMV seropositivity. Valganciclovir for CMV suppression is also not required in this case. Fungal prophylaxis is essential to prevent invasive fungal infections in high-risk patients like this 4-year-old with leukemia.

Correct Answer: C

Rationale: The correct answer is C: She has an excellent prognosis with surgery, chemotherapy, and radiation. This statement is not true because malignant rhabdoid tumor of the kidney (MRTK) has a poor prognosis, even with aggressive treatment. Here's a breakdown: 1. MRTK is an aggressive and rare tumor that often presents in infancy, supporting statement A. 2. Most patients with MRTK present with metastatic disease, indicating poor prognosis, aligning with statement B. 3. Germline testing for SMARCB1/INI1 mutation is essential due to the genetic predisposition associated with MRTK, supporting statement D. In summary, statement C is incorrect as MRTK typically has a challenging clinical course despite comprehensive treatment approaches.

Correct Answer: D

Rationale: The correct answer is D: Growth hormone deficiency due to iron deposition in the pituitary. Iron deposition in the pituitary gland can lead to impaired production and secretion of growth hormone, resulting in short stature. This is a known complication of beta thalassemia major. The patient's elevated serum ferritin levels and T2* value of 9 ms indicate iron overload, which can cause damage to organs including the pituitary gland. Choices A, B, and C are incorrect because the patient's growth failure is primarily due to the impact of iron deposition on the pituitary gland rather than lack of transfusion, cirrhosis, or ineffective erythropoiesis. Regular chelation therapy is essential to prevent iron overload and its complications in patients with beta thalassemia major.

Correct Answer: D

Rationale: The correct answer is D: Ristoectin cofactor activity. This patient's history of significant menstrual bleeding, epistaxis, and low hemoglobin suggests a congenital bleeding disorder. Ristoectin cofactor activity is a test for von Willebrand disease (VWD), a common inherited bleeding disorder characterized by a deficiency or dysfunction of von Willebrand factor. VWD typically presents with mucocutaneous bleeding, such as epistaxis and menorrhagia. Choice A: Factor XI level is not the most likely diagnosis as Factor XI deficiency (Hemophilia C) typically presents with bleeding after surgery or trauma, not mucocutaneous bleeding. Choice B: Factor X level is not the most likely diagnosis as Factor X deficiency presents with bleeding that is more severe and can cause hemarthrosis, not typically seen in this patient. Choice C: Factor XIII level is not the most likely diagnosis as Factor XIII deficiency leads to delayed wound healing and poor clot formation,