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. In this case, the patient is at high risk for fungal infections post-transplant due to immunosuppression from the conditioning regimen. The patient is negative for CMV IgG and IgM, so CMV prophylaxis is not necessary (eliminating choices B and D). The patient is also negative for HSV antibodies, so acyclovir for HSV suppression is not indicated (eliminating choice A). Therefore, antifungal prophylaxis is the most appropriate choice to prevent fungal infections in this immunocompromised patient. It is essential to protect the patient from opportunistic infections, and antifungal prophylaxis is a crucial component of post-transplant care.
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 overload in patients with beta thalassemia major can lead to iron deposition in various organs, including the pituitary gland, impairing its function. This can result in growth hormone deficiency, leading to short stature. In this case, the patient's history of chronic transfusions and high serum ferritin level indicate iron overload, which can affect the pituitary gland. Choices A, B, and C are incorrect because growth failure in beta thalassemia major is primarily attributed to endocrine complications such as growth hormone deficiency, rather than lack of transfusions, cirrhosis, or ineffective erythropoiesis.
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. 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 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 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 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: 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.