ATI RN
ATI Hematologic System Questions
Question 1 of 5
A 3-month-old female presents to the emergency room with vomiting and abdominal distension. She has a left-side abdominal mass, and an abdominal ultrasound confirms an 8-cm mass arising from the left kidney. Liver lesions are also noted. Nephrectomy is performed and reveals a histologic diagnosis of malignant rhabdoid tumor of the kidney (MRTK). Which of the following is not a true statement about the management of this patient?
Correct Answer: C
Rationale: The correct answer is C: She has an excellent prognosis with surgery, chemotherapy, and radiation. This statement is not true because malignant rhabdoid tumor of the kidney (MRTK) has a poor prognosis, even with aggressive treatment. Here's a breakdown: 1. MRTK is an aggressive and rare tumor that often presents in infancy, supporting statement A. 2. Most patients with MRTK present with metastatic disease, indicating poor prognosis, aligning with statement B. 3. Germline testing for SMARCB1/INI1 mutation is essential due to the genetic predisposition associated with MRTK, supporting statement D. In summary, statement C is incorrect as MRTK typically has a challenging clinical course despite comprehensive treatment approaches.
Question 2 of 5
A 12-year-old girl presents to your clinic with significant menstrual bleeding at the onset of menarche and is noted to have a hemoglobin of 9.9, although she is not symptomatic from her anemia. Her mother reports that she has a history of epistaxis when she was a child with some episodes lasting 30 minutes and that she also has heavy menstrual bleeding. Which of the following tests will lead to the most likely diagnosis?
Correct Answer: D
Rationale: The correct answer is D: Ristoectin cofactor activity. This patient's history of significant menstrual bleeding, epistaxis, and low hemoglobin suggests a congenital bleeding disorder. Ristoectin cofactor activity is a test for von Willebrand disease (VWD), a common inherited bleeding disorder characterized by a deficiency or dysfunction of von Willebrand factor. VWD typically presents with mucocutaneous bleeding, such as epistaxis and menorrhagia. Choice A: Factor XI level is not the most likely diagnosis as Factor XI deficiency (Hemophilia C) typically presents with bleeding after surgery or trauma, not mucocutaneous bleeding. Choice B: Factor X level is not the most likely diagnosis as Factor X deficiency presents with bleeding that is more severe and can cause hemarthrosis, not typically seen in this patient. Choice C: Factor XIII level is not the most likely diagnosis as Factor XIII deficiency leads to delayed wound healing and poor clot formation,
Question 3 of 5
You receive a phone call that a 3-year-old patient on long-term warfarin therapy for congenital heart disease has an international normalized ratio (INR) of 5.8. On further history, you learn the patient and several family members have had recent gastrointestinal illnesses, but the patient is recovering. His mother reports he is not experiencing bleeding symptoms. Which of the following interventions would be most reasonable in this clinical scenario?
Correct Answer: A
Rationale: The correct answer is A: Hold 1 to 2 doses of warfarin and recheck INR. In this scenario, the high INR indicates an increased risk of bleeding due to excess anticoagulation. However, since the patient is asymptomatic and the high INR is likely due to transient factors (gastrointestinal illness), the most appropriate initial step is to temporarily hold warfarin to allow the INR to normalize. Rechecking the INR after holding the doses will guide further management. Choice B (Administer oral vitamin K therapy) is incorrect because the patient is not experiencing bleeding symptoms and oral vitamin K should be reserved for patients with active bleeding or high INR with bleeding symptoms. Choice C (Administer fresh frozen plasma) is inappropriate as the patient is not actively bleeding and FFP is typically reserved for acute bleeding situations. Choice D (Administer recombinant factor VIIa) is also incorrect as it is reserved for severe bleeding in patients on war
Question 4 of 5
Assuming that adherence has been excellent, which of the following should have returned to normal 6 weeks following appropriate oral iron treatment for a child with severe dietary iron deficiency (hemoglobin [Hgb] 5.0 g/dL and mean corpuscular volume [MCV] 48 fL at the beginning of therapy)?
Correct Answer: A
Rationale: Rationale for Correct Answer (A): Hgb concentration reflects the amount of hemoglobin in the blood, which is directly affected by iron levels. After 6 weeks of appropriate oral iron treatment, the child's severe iron deficiency should have improved, leading to an increase in Hgb concentration back to normal levels. Explanation of Incorrect Choices: B: MCV measures the size of red blood cells, which is affected by iron deficiency anemia. However, it may take longer than 6 weeks for MCV to return to normal. C: Red cell distribution width indicates the variation in red blood cell sizes and is not directly related to iron levels. It may not necessarily return to normal within 6 weeks. D: Peripheral blood smear provides information on the morphology of red blood cells but does not directly reflect iron levels. It may not show significant changes within 6 weeks of iron treatment.
Question 5 of 5
You have a new 7-year-old female patient with a WBC count of 6,000/mm3, hemoglobin of 7.2 g/dL, and platelet count of 30,000/mm3. A bone marrow aspirate reveals 14% blasts with a monocytic morphologic appearance that are surface marker positive for CD33. You receive a call from the fluorescence in situ hybridization (FISH) lab that the bone marrow is positive for KMT2A rearrangement in 68% of cells. Your staff asks whether this represents a diagnosis of acute leukemia in the current classification scheme for this type of hematologic malignancy. What would you say?
Correct Answer: D
Rationale: The correct answer is D: Yes, because the FISH is positive for KMT2A rearrangement. Rationale: 1. KMT2A rearrangement is a genetic abnormality commonly associated with acute leukemia. 2. Presence of blasts (14%) with monocytic appearance and positive for CD33 also supports the diagnosis. 3. The percentage of blasts (14%) is not below the threshold for acute leukemia diagnosis. 4. The specific cytogenetic findings mentioned in choice C are not absolute requirements for diagnosing acute leukemia. In summary, the presence of KMT2A rearrangement, along with morphologic and flow cytometry findings, supports the diagnosis of acute leukemia in this case, making choice D the correct answer.