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 scenario is Juvenile myelomonocytic leukemia (JMML). This is supported by the presence of hepatosplenomegaly, maculopapular rash, leukocytosis, anemia, and thrombocytopenia in a young child. The elevated fetal hemoglobin level is characteristic of JMML. Additionally, a normal blood karyotype helps differentiate JMML from other leukemias. Choice A (Leukemoid Reaction) is incorrect because it is typically a reactive condition due to infections, not a primary hematological malignancy like JMML. Choice B (Acute lymphoblastic leukemia) is less likely due to the presence of hepatosplenomegaly and a high fetal hemoglobin level. Choice C (Chronic myeloid leukemia) is less likely in a young child with the given clinical presentation.
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 history, including a family member with aplastic anemia and a cousin with leukemia, raises suspicion for a genetic disorder like Fanconi anemia. Testing skin fibroblasts for Fanconi anemia can help confirm or rule out this diagnosis. Administering ATG and cyclosporine (choice A) may not be effective if the underlying cause is a genetic disorder. Searching for a donor for matched unrelated transplant (choice B) is premature without confirming the diagnosis. Sending a bone marrow aspirate for Fanconi anemia testing (choice C) may not yield accurate results as the peripheral blood sample test was negative, making skin fibroblast culture the preferred choice.
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: 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.
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: The correct answer is C: Interleukin-1 receptor-associated kinase 4 (IRAK4). 1. IRAK4 is involved in the immune response pathway triggered by interleukin-1 receptor signaling. 2. Lack of Toll-like receptor responses in the patient aligns with the role of IRAK4 in the interleukin-1 receptor pathway. 3. Pathogenic variants in IRAK4 can lead to immunodeficiency, explaining recurrent Staph infections. 4. Choices A, B, and D do not accurately reflect the known function of IRAK4 and its association with interleukin-1 receptor signaling.
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. 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.