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 because the patient is in morphologic remission and MRD-negative after the second course of treatment. Given her high-risk AML and lack of HLA-matched siblings, a matched unrelated donor HSCT offers the best chance for long-term remission. Autologous HSCT (choice B) would risk reintroducing leukemic cells. Giving more courses of intensification chemotherapy alone (choice A) may not eradicate the disease. Maintenance chemotherapy alone (choice D) is not sufficient for high-risk AML. Therefore, choice C is the best option for maximizing the chances of a favorable outcome in this case.
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. A platelet count of 1,200,000/mm3 suggests thrombocytosis, which can be seen in conditions like essential thrombocythemia. This condition is associated with low von Willebrand factor activity, leading to increased bleeding tendency despite high platelet counts. Numbness, tingling, epistaxis, and splenomegaly are common features of essential thrombocythemia. Elevated C-reactive protein is not typically associated with this condition. Low ferritin is more indicative of iron deficiency anemia, not thrombocytosis. A hypocellular bone marrow is not expected in essential thrombocythemia, as it is characterized by increased platelet production.
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 correct answer is C: Perform emergent leukapheresis plus hydroxyurea. In this scenario, the patient presents with symptoms suggestive of acute myeloid leukemia with hyperleukocytosis and intracerebral hemorrhage. The goal of emergent therapy is to rapidly reduce the high blast count to prevent further complications such as leukostasis and hemorrhage. Leukapheresis can provide immediate reduction in the blast count, while hydroxyurea can further decrease the WBC count. Starting induction chemotherapy immediately can lead to tumor lysis syndrome due to rapid cell destruction. Emergent cranial radiation is not the primary intervention for hyperleukocytosis. Performing leukapheresis alone without adjunctive therapy like hydroxyurea may not adequately control the blast count. Thus, the most appropriate initial therapy is emergent leukapheresis plus hydroxyurea to stabilize the patient before initiating induction chemotherapy.
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. This aligns with the six dimensions of quality care outlined by the Institute of Medicine. Safety ensures patient well-being, timeliness reduces delays, effectiveness means providing evidence-based care, efficiency minimizes waste, equity ensures fair access, and person-centered care considers individual preferences. Choice B is incorrect because transparency is not included in the Institute of Medicine's six dimensions. Choice C is incorrect as "low-cost" is not a recognized dimension of quality care. Choice D is incorrect because "cutting-edge" is not part of the established dimensions of quality care.
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 correct answer is C: Chronic myeloid leukemia (CML). This is a likely diagnosis due to the presence of fatigue, weight loss, night sweats, splenomegaly, anemia, thrombocytosis, and leukocytosis with an elevated white blood cell count. CML is characterized by the Philadelphia chromosome, resulting in the BCR-ABL fusion gene. This gene leads to uncontrolled proliferation of myeloid cells, causing the symptoms seen in this patient. Leukemoid reaction (A) is a reactive increase in white blood cells, usually in response to an infection. Acute lymphoblastic leukemia (B) typically presents with symptoms in children but is more commonly associated with lymphoblasts in the peripheral blood. JMML (D) is a rare myelodysplastic disorder in children characterized by monocytosis and a specific genetic mutation.