ATI RN
ATI Hematologic System Questions
Question 1 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 2 of 5
A 4-year-old male child presents to the emergency department with his fourth invasive Staph infection. CBC consistently identifies moderate neutropenia. Sophisticated lab testing identifies lack of Toll-like receptor responses. The patient undergoes whole exome sequencing and is found to have pathogenic variants in IRAK4. What does 'IRAK4' stand for?
Correct Answer: C
Rationale: Step 1: Identify the function of IRAK4. IRAK4 is involved in the signaling pathway of the immune system, particularly in response to interleukin-1 (IL-1) receptor activation. Step 2: Break down the acronym IRAK4. IRAK4 stands for Interleukin-1 Receptor-Associated Kinase 4. Step 3: Link the information in the question to the correct answer. Given that the patient has a lack of Toll-like receptor responses and pathogenic variants in IRAK4, it indicates a problem with the interleukin-1 signaling pathway, making choice C (Interleukin-1 receptor-associated kinase 4) the correct answer. Summary: A: Incorrect - Interferon gamma receptor-associated kinase does not match the function of IRAK4. B: Incorrect - Inducible RAS activating kinase does not match the function of IRAK4. C: Correct - Matches the function of IRAK4
Question 3 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 4 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 5 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.