ATI RN
ATI Hematologic System Questions
Question 1 of 5
A 4-year-old girl with a history of relapsed pre-B-cell acute lymphoblastic leukemia is being admitted for unrelated donor bone marrow transplantation with cyclophosphamide and total body irradiation conditioning. Pretransplant workup shows the following: Recipient: CMV IgG: negative, CMV IgM: negative, HSV I/II antibody: negative, Varicella IgG: positive (vaccinated), Hepatitis B surface antigen: negative, Hepatitis B surface antibody: positive (vaccinated), Hepatitis B core antibody: negative, Hepatitis C antibody: negative. Donor: CMV IgG: negative, CMV IgM: negative, HSV I/II antibody: positive, Varicella IgG: positive, Hepatitis B surface antigen: negative, Hepatitis B core antibody: negative, Hepatitis C antibody: negative. How should the patient be managed during the admission with respect to infection prophylaxis?
Correct Answer: C
Rationale: The correct answer is C: Antifungal prophylaxis. This patient is at high risk for fungal infections post-bone marrow transplantation due to the conditioning regimen with cyclophosphamide and total body irradiation. CMV and HSV prophylaxis are not indicated as the patient and donor are negative for CMV IgG and IgM and the donor is positive for HSV antibodies. Weekly CMV PCR screening is not necessary in the absence of CMV seropositivity. Valganciclovir for CMV suppression is also not required in this case. Fungal prophylaxis is essential to prevent invasive fungal infections in high-risk patients like this 4-year-old with leukemia.
Question 2 of 5
A 14-year-old Syrian male with beta thalassemia major has relocated to your community as a refugee. He has been receiving chronic transfusion therapy in Turkey for the past 3 years. On his first visit, you notice that his height is below the fifth percentile. He has skin discoloration and hepatosplenomegaly. His mother reports they have not had regular access to chelation therapy. Laboratory testing shows a serum ferritin of 6,200 ng/mL. A cardiac MRI shows grossly normal cardiac function but a T2* value of 9 ms. What is the most likely cause of his short stature?
Correct Answer: D
Rationale: The correct answer is D: Growth hormone deficiency due to iron deposition in the pituitary. Iron deposition in the pituitary gland can lead to impaired production and secretion of growth hormone, resulting in short stature. This is a known complication of beta thalassemia major. The patient's elevated serum ferritin levels and T2* value of 9 ms indicate iron overload, which can cause damage to organs including the pituitary gland. Choices A, B, and C are incorrect because the patient's growth failure is primarily due to the impact of iron deposition on the pituitary gland rather than lack of transfusion, cirrhosis, or ineffective erythropoiesis. Regular chelation therapy is essential to prevent iron overload and its complications in patients with beta thalassemia major.
Question 3 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 4 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 5 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.