ATI RN
Anatomy of Hematologic System Questions
Question 1 of 5
A 13-year-old girl presents with acute myeloid leukemia (AML) and a WBC count of 120,000/mm3. Cytogenetics reveals a normal karyotype, and fluorescence in situ hybridization (FISH) tests for inv(16), t(8;21), t(15;17); 11q23 abnormalities; monosomy 7; and 5q deletion are negative. Molecular testing is negative for mutations in FLT3, NPM1, and CEBPA. She is treated with 10 days of daunorubicin, AraC, and gemtuzumab for induction therapy. On day 30, she recovers counts, and a bone marrow aspiration shows 2.2% leukemic blasts by flow cytometry. She receives a second course of treatment with daunorubicin and AraC, and her marrow is now in morphologic remission and is MRD-negative by flow cytometry. She has no HLA-matched siblings, but an unrelated donor search reveals a large number of potential matches. Which course of treatment is most likely to result in the best outcome?
Correct Answer: C
Rationale: The correct answer is C: Give one more course of intensification chemotherapy and then perform a matched unrelated donor HSCT. Rationale: 1. The patient achieved morphologic remission and MRD-negative status after the second course of chemotherapy, indicating good response. 2. Given the high-risk AML with negative cytogenetics and molecular markers, HSCT from a matched unrelated donor offers the best chance for long-term remission. 3. HSCT provides a curative option by replacing the patient's diseased bone marrow with healthy donor cells, reducing the risk of relapse. 4. The presence of a large number of potential matched unrelated donors increases the likelihood of finding a suitable donor for the transplant. Summary: - Option A: Giving more courses of intensification chemotherapy may not address the high-risk nature of the disease and may not provide a curative outcome. - Option B: Autologous HSCT uses the patient's own stem cells, which may carry the risk of relapse due
Question 2 of 5
A 17-year-old patient is referred to you for a platelet count of 1,200,000/mm3. On history, she notes that she often has numbness and tingling in her hands and feet and has frequent epistaxis. She is otherwise well-appearing and has no recent infections. On her exam, you note splenomegaly. What do you expect to see on further evaluation?
Correct Answer: D
Rationale: The correct answer is D: Low von Willebrand factor activity. This patient likely has Essential Thrombocythemia, a disorder characterized by excessive production of platelets by the bone marrow. This condition can lead to abnormal bleeding and clotting. The patient's symptoms of numbness, tingling, epistaxis, and splenomegaly are consistent with this diagnosis. Low von Willebrand factor activity is expected in Essential Thrombocythemia due to platelet dysfunction. Elevated C-reactive protein, low ferritin, and a hypocellular bone marrow are not typically associated with Essential Thrombocythemia and are less likely to be seen in this patient.
Question 3 of 5
A 13-year-old boy presents to the emergency department with complaints of headache and visual changes. History reveals progressive dyspnea on exertion, generalized fatigue, and increased bruising. His labs are significant for a WBC of 350,000/mcL, of which 80% are reported to be blasts and appear to be myeloblasts without the presence of Auer rods. His hemoglobin is 7.2 g/dL, and his platelets are 18,000/mcL. A CT scan of the head shows a small intracerebral hemorrhage. His coags are normal. Which of the following is the most appropriate therapy?
Correct Answer: C
Rationale: The most appropriate therapy in this scenario is emergent leukapheresis plus hydroxyurea (Choice C). Leukapheresis is needed to rapidly reduce the high white blood cell count, preventing further complications such as leukostasis and hyperviscosity syndrome. Hydroxyurea can help further control the rapid proliferation of blasts. Induction chemotherapy (Choice A) may lead to tumor lysis syndrome due to the high tumor burden. Performing leukapheresis alone without a subsequent therapy (Choice B) may not adequately address the underlying disease. Providing cranial radiation (Choice D) is not the initial management for acute myeloid leukemia.
Question 4 of 5
What are the six dimensions of quality care according to the Institute of Medicine?
Correct Answer: A
Rationale: The correct answer is A: Safe, timely, effective, efficient, equitable, and person-centered. According to the Institute of Medicine, these six dimensions encompass quality care. Safety is essential to avoid harm. Timeliness ensures care is provided promptly. Effectiveness means care should be evidence-based and achieve desired outcomes. Efficiency aims to minimize waste. Equitability ensures fair and non-discriminatory care. Lastly, being person-centered emphasizes individual preferences and needs. Summary: Choice B is incorrect because transparency is not included in the six dimensions. Choice C is incorrect as low-cost is not a primary dimension of quality care. Choice D is incorrect as it includes cutting-edge, which is not one of the six dimensions outlined by the Institute of Medicine.
Question 5 of 5
You are seeing a 13-year-old boy with fatigue, weight loss, night sweats, and splenomegaly. Peripheral blood shows anemia, thrombocytosis, and leukocytosis (300,000/mm3). What is this patient's most likely diagnosis?
Correct Answer: C
Rationale: The most likely diagnosis for this 13-year-old boy presenting with fatigue, weight loss, night sweats, splenomegaly, anemia, thrombocytosis, leukocytosis (300,000/mm3) is Chronic Myeloid Leukemia (CML). 1. Presence of anemia, thrombocytosis, and leukocytosis is characteristic of CML. 2. CML typically presents with splenomegaly. 3. Age of onset (13 years) is within the typical range for CML. 4. The Philadelphia chromosome (t(9;22)) is present in most cases of CML, aiding in diagnosis. 5. Leukemoid reaction (A) is a reactive increase in white blood cells, not a primary neoplastic disorder. 6. Acute lymphoblastic leukemia (ALL) (B) typically presents with bone pain, lymphadenopathy, and CNS symptoms in children. 7