ATI RN
ATI Hematologic System Questions
Question 1 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).
Question 2 of 5
A 4-year-old girl with a history of relapsed pre-B-cell acute lymphoblastic leukemia is being admitted for unrelated donor bone marrow transplantation with cyclophosphamide and total body irradiation conditioning. Pretransplant workup shows the following: Recipient: CMV IgG: negative, CMV IgM: negative, HSV I/II antibody: negative, Varicella IgG: positive (vaccinated), Hepatitis B surface antigen: negative, Hepatitis B surface antibody: positive (vaccinated), Hepatitis B core antibody: negative, Hepatitis C antibody: negative. Donor: CMV IgG: negative, CMV IgM: negative, HSV I/II antibody: positive, Varicella IgG: positive, Hepatitis B surface antigen: negative, Hepatitis B core antibody: negative, Hepatitis C antibody: negative. How should the patient be managed during the admission with respect to infection prophylaxis?
Correct Answer: C
Rationale: The correct answer is C: Antifungal prophylaxis. In this case, the patient is at high risk for fungal infections post-transplant due to immunosuppression from the conditioning regimen. The patient is negative for CMV IgG and IgM, so CMV prophylaxis is not necessary (eliminating choices B and D). The patient is also negative for HSV antibodies, so acyclovir for HSV suppression is not indicated (eliminating choice A). Therefore, antifungal prophylaxis is the most appropriate choice to prevent fungal infections in this immunocompromised patient. It is essential to protect the patient from opportunistic infections, and antifungal prophylaxis is a crucial component of post-transplant care.
Question 3 of 5
A 3-month-old female presents to the emergency room with vomiting and abdominal distension. She has a left-side abdominal mass, and an abdominal ultrasound confirms an 8-cm mass arising from the left kidney. Liver lesions are also noted. Nephrectomy is performed and reveals a histologic diagnosis of malignant rhabdoid tumor of the kidney (MRTK). Which of the following is not a true statement about the management of this patient?
Correct Answer: C
Rationale: The correct answer is C: She has an excellent prognosis with surgery, chemotherapy, and radiation. Rationale: 1. Malignant rhabdoid tumor of the kidney (MRTK) is an aggressive cancer with poor prognosis. 2. Even with aggressive treatment, including surgery, chemotherapy, and radiation, the prognosis is generally poor due to high rates of recurrence and metastasis. 3. Therefore, stating that the patient has an excellent prognosis with the mentioned treatments is not true. Summary of other choices: A: Most patients with rhabdoid tumor of the kidney present in infancy - True, MRTK commonly presents in infancy. B: Most patients with rhabdoid tumor of the kidney present with metastatic (stage III or IV) disease - True, MRTK often presents with metastatic disease. D: Germline testing for SMARCB1/INI1 mutation on chromosome 22 is recommended, with brain MRI every 3 months until she is
Question 4 of 5
A 14-year-old Syrian male with beta thalassemia major has relocated to your community as a refugee. He has been receiving chronic transfusion therapy in Turkey for the past 3 years. On his first visit, you notice that his height is below the fifth percentile. He has skin discoloration and hepatosplenomegaly. His mother reports they have not had regular access to chelation therapy. Laboratory testing shows a serum ferritin of 6,200 ng/mL. A cardiac MRI shows grossly normal cardiac function but a T2* value of 9 ms. What is the most likely cause of his short stature?
Correct Answer: D
Rationale: The correct answer is D: Growth hormone deficiency due to iron deposition in the pituitary. Iron overload in patients with beta thalassemia major can lead to iron deposition in various organs, including the pituitary gland, impairing its function. This can result in growth hormone deficiency, leading to short stature. In this case, the patient's history of chronic transfusions and high serum ferritin level indicate iron overload, which can affect the pituitary gland. Choices A, B, and C are incorrect because growth failure in beta thalassemia major is primarily attributed to endocrine complications such as growth hormone deficiency, rather than lack of transfusions, cirrhosis, or ineffective erythropoiesis.
Question 5 of 5
A 12-year-old girl presents to your clinic with significant menstrual bleeding at the onset of menarche and is noted to have a hemoglobin of 9.9, although she is not symptomatic from her anemia. Her mother reports that she has a history of epistaxis when she was a child with some episodes lasting 30 minutes and that she also has heavy menstrual bleeding. Which of the following tests will lead to the most likely diagnosis?
Correct Answer: D
Rationale: The correct answer is D: Ristoectin cofactor activity. The scenario describes a young girl with significant menstrual bleeding and a history of prolonged epistaxis, suggesting a bleeding disorder. Given the clinical presentation, the most likely diagnosis is von Willebrand disease (vWD), the most common inherited bleeding disorder. Ristocetin cofactor activity is a specific test for vWD, as it assesses the ability of von Willebrand factor to bind to platelets in the presence of ristocetin. Factors XI, X, and XIII are not typically associated with vWD, making choices A, B, and C incorrect. Testing for Factor VIII/von Willebrand factor antigen levels and vWF multimer analysis may also be considered to confirm the diagnosis.