ATI RN
ATI Hematologic System Questions
Question 1 of 5
A 13-year-old Hispanic girl is found to have a WBC count of 6,500/mm3 with 40% Auer rod–containing granular blasts that, by flow cytometry, express very bright CD33 but are negative for human leukocyte antigen–DR isotype (HLA-DR). She is oozing blood around her peripheral IV site. Coagulation studies reveal an international normalized ratio (INR) of 3.4, a fibrinogen of 170, and a markedly elevated D-dimer. Marrow aspirate shows nearly 90% blasts with a similar morphology. You send the marrow to the fluorescence in situ hybridization (FISH) lab and request STAT testing for the most likely recurrent genetic abnormality based on the clinical presentation. How do you plan to initiate therapy?
Correct Answer: B
Rationale: The correct answer is B because the clinical presentation suggests acute promyelocytic leukemia (APL). APL is characterized by Auer rod-containing granular blasts, which are positive for CD33 and negative for HLA-DR. The presence of coagulopathy with elevated D-dimer and oozing blood indicates a high risk of disseminated intravascular coagulation (DIC), a common complication of APL. Immediate treatment with all-trans retinoic acid (ATRA) is crucial in APL to induce differentiation of the leukemic cells and prevent further coagulopathy. Aggressively managing the coagulopathy with blood product support is also important to stabilize the patient. Choice A is incorrect because a lumbar puncture is not necessary for APL diagnosis, and immediate induction chemotherapy with ATRA is the standard of care. Choice C is incorrect because dexamethasone and hydroxyurea are not the first-line therapies for APL.
Question 2 of 5
Which of the following statements about myeloablative, myeloablative but reduced toxicity, reduced intensity, and non-myeloablative approaches is not correct?
Correct Answer: B
Rationale: The correct answer is B because reduced intensity regimens are used to minimize toxicity and late effects, not for most nonmalignant disorders. Myeloablative approaches are needed for high-risk malignancies to maximize remission depth (A), reduced intensity regimens can increase transplant-related mortality in high-risk patients (C), and non-myeloablative regimens are used for the highest risk patients and certain diseases like aplastic anemia (D).
Question 3 of 5
A 4-year-old male child presents to the emergency department with his fourth invasive Staph infection. CBC consistently identifies moderate neutropenia. Sophisticated lab testing identifies lack of Toll-like receptor responses. The patient undergoes whole exome sequencing and is found to have pathogenic variants in IRAK4. What does 'IRAK4' stand for?
Correct Answer: C
Rationale: Step 1: Identify the function of IRAK4. IRAK4 is involved in the signaling pathway of the immune system, particularly in response to interleukin-1 (IL-1) receptor activation. Step 2: Break down the acronym IRAK4. IRAK4 stands for Interleukin-1 Receptor-Associated Kinase 4. Step 3: Link the information in the question to the correct answer. Given that the patient has a lack of Toll-like receptor responses and pathogenic variants in IRAK4, it indicates a problem with the interleukin-1 signaling pathway, making choice C (Interleukin-1 receptor-associated kinase 4) the correct answer. Summary: A: Incorrect - Interferon gamma receptor-associated kinase does not match the function of IRAK4. B: Incorrect - Inducible RAS activating kinase does not match the function of IRAK4. C: Correct - Matches the function of IRAK4
Question 4 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 5 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