ATI RN
ATI Hematologic System Questions
Question 1 of 5
You are seeing a 2-year-old girl with new onset of fever and bronchitis. She has maculopapular rash and hepatosplenomegaly. Blood smear shows leukocytosis (100,000/mm3), anemia, and thrombocytopenia. Ancillary tests include fetal hemoglobin of 80% and normal blood karyotype. What is the most likely diagnosis?
Correct Answer: D
Rationale: The most likely diagnosis in this case is Juvenile myelomonocytic leukemia (JMML). JMML is a rare myelodysplastic/myeloproliferative neoplasm seen in young children. The clinical presentation of fever, rash, hepatosplenomegaly, leukocytosis, anemia, and thrombocytopenia is consistent with JMML. The presence of fetal hemoglobin of 80% is a key finding in JMML, as it is a distinguishing feature. Additionally, a normal blood karyotype rules out chromosomal abnormalities commonly seen in other leukemias. Leukemoid reaction (Choice A) is characterized by a reactive increase in leukocyte count due to an underlying condition, but it does not explain the other findings in this case. Acute lymphoblastic leukemia (ALL - Choice B) primarily affects lymphoid cells, not myeloid cells as seen in this case. Chronic myeloid leukemia (C
Question 2 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 3 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 4 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 5 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.