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. Rationale: 1. Malignant rhabdoid tumor of the kidney (MRTK) is an aggressive cancer with poor prognosis. 2. Even with aggressive treatment, including surgery, chemotherapy, and radiation, the prognosis is generally poor due to high rates of recurrence and metastasis. 3. Therefore, stating that the patient has an excellent prognosis with the mentioned treatments is not true. Summary of other choices: A: Most patients with rhabdoid tumor of the kidney present in infancy - True, MRTK commonly presents in infancy. B: Most patients with rhabdoid tumor of the kidney present with metastatic (stage III or IV) disease - True, MRTK often presents with metastatic disease. D: Germline testing for SMARCB1/INI1 mutation on chromosome 22 is recommended, with brain MRI every 3 months until she is
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. The scenario describes a young girl with significant menstrual bleeding and a history of prolonged epistaxis, suggesting a bleeding disorder. Given the clinical presentation, the most likely diagnosis is von Willebrand disease (vWD), the most common inherited bleeding disorder. Ristocetin cofactor activity is a specific test for vWD, as it assesses the ability of von Willebrand factor to bind to platelets in the presence of ristocetin. Factors XI, X, and XIII are not typically associated with vWD, making choices A, B, and C incorrect. Testing for Factor VIII/von Willebrand factor antigen levels and vWF multimer analysis may also be considered to confirm the diagnosis.
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 patient's elevated INR of 5.8 indicates an increased risk of bleeding due to excessive anticoagulation. Since the patient is not experiencing bleeding symptoms and is recovering from gastrointestinal illness, temporarily holding 1 to 2 doses of warfarin is the most reasonable intervention to prevent bleeding complications while allowing the INR to normalize. Rechecking the INR after holding the doses will help assess the patient's response to the intervention. Choices B, C, and D are incorrect because administering oral vitamin K therapy, FFP, or recombinant factor VIIa are more aggressive interventions that are not warranted in this case where the patient is asymptomatic and recovering from a transient illness.
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: 1. Hemoglobin (Hgb) reflects the oxygen-carrying capacity of red blood cells. 2. Severe dietary iron deficiency causes low Hgb levels. 3. Adequate iron treatment should lead to increased Hgb levels. 4. MCV and other parameters may take longer to normalize. Summary: B, C, and D are incorrect because MCV and red cell distribution width may take longer to normalize, and peripheral blood smear changes may persist even after Hgb has returned to normal.
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. The presence of KMT2A rearrangement in 68% of cells indicates a specific genetic abnormality associated with acute leukemia. This abnormality is a known marker for acute myeloid leukemia (AML) with recurrent genetic abnormalities. The percentage of blasts in the bone marrow (14%) may not meet the traditional criteria for a diagnosis of acute leukemia based on blast percentage alone, but the presence of KMT2A rearrangement overrides this requirement in this case. Choices A and B are incorrect because blast percentage alone is not the sole determinant of a diagnosis of acute leukemia when specific genetic abnormalities are present. Choice C is incorrect because while the absence of specific cytogenetic abnormalities may be relevant for some cases, the presence of KMT2A rearrangement is sufficient to support a diagnosis of acute leukemia in this context.