ATI RN
Anatomy of Hematologic System Questions
Question 1 of 5
A 10-year-old girl is a long-term survivor of type II pleuropulmonary blastoma (PPB). You suspect she has a cancer predisposition syndrome and perform genetic testing, which confirms she has DICER1 syndrome. Which other cancer is she predisposed to?
Correct Answer: A
Rationale: The correct answer is A: Papillary thyroid cancer. Individuals with DICER1 syndrome are at increased risk for developing various tumors, including papillary thyroid cancer. This is due to the role of DICER1 gene in regulating cell growth and division. The other choices, B (medullary thyroid cancer), C (pheochromocytoma), and D (renal cell carcinoma), are not typically associated with DICER1 syndrome. Medullary thyroid cancer is commonly linked to RET gene mutations, pheochromocytoma to genes such as SDHB, SDHD, and VHL, and renal cell carcinoma to mutations in genes like VHL and MET.
Question 2 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 3 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 4 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.
Question 5 of 5
An infant is born with a 7 cm × 6 cm lesion over the upper extremity from the elbow to the shoulder. The lesion is indurated and purpuric, with some petechiae around the edges. No other areas of petechiae are noted on the skin. The infant is doing well without other systemic problems. i were 9 and 9. You are called by the pediatric nurse practitioner to the NICU. What is the most appropriate next step?
Correct Answer: D
Rationale: The correct next step is D: Obtain labs, including a CBC with platelet count and fibrinogen. This is the most appropriate action because the infant presents with an indurated and purpuric lesion with petechiae, suggesting a possible coagulation disorder. Labs can help assess for any underlying hematologic abnormalities. Choice A is incorrect because it is important to investigate further given the concerning presentation. Choice B is not the best next step as an ultrasound would not provide information on the hematologic status. Choice C is also not the best option as an MRI is not typically used to assess coagulation disorders.