ATI RN
Hematological System Questions
Question 1 of 5
An avid 16-year-old triathlete was in a bike accident and developed cellulitis, which was treated with Bactrim. While still on antibiotics, he moved with his family from Houston to Denver, and during the car trip he developed fever, pharyngitis, and malaise. Upon arriving in Denver he presented to the emergency department and was noted to have significant lymphocytosis with some atypical lymphocytes. What is the most likely cause of the white blood cell abnormalities?
Correct Answer: D
Rationale: The correct answer is D: Epstein-Barr virus infection. The presentation of fever, pharyngitis, malaise, lymphocytosis, and atypical lymphocytes is classic for infectious mononucleosis caused by Epstein-Barr virus. The patient's symptoms started after the bike accident and while on antibiotics, suggesting a new infectious etiology. This viral infection commonly causes lymphocytosis with atypical lymphocytes. Choice A (Drug reaction) is less likely as the symptoms started after the antibiotics were initiated. Choice B (Altitude higher than 5,000 ft above sea level) is unlikely to cause these specific white blood cell abnormalities. Choice C (GATA2 mutation) is less likely as there are no indications in the scenario to suggest a genetic disorder.
Question 2 of 5
A 3-year-old nonsyndromic, well-appearing male with no significant past medical history presents with an abdominal mass palpated by his mother when giving him a bath. CT imaging reveals a 9-cm right renal mass without involvement of the inferior vena cava (IVC) and no evidence of tumor thrombus by ultrasound. The left kidney appears normal, and there is no imaging evidence of tumor rupture or adherence to surrounding organs. There are diffuse, bilateral pulmonary metastases from which he is asymptomatic with a normal respiratory rate and no supplemental oxygen requirement. Following the National Wilms Tumor Study Group (NWTS)/Children's Oncology Group (COG) approach to pediatric renal tumors, which of the following are appropriate next steps?
Correct Answer: B
Rationale: The correct answer is B: Nephrectomy with lymph node sampling followed by chemotherapy based on histology and stage. This is the appropriate next step following NWTS/COG approach for pediatric renal tumors. Nephrectomy is crucial to remove the primary tumor and obtain tissue for definitive histologic diagnosis. Lymph node sampling helps determine if there is spread to regional nodes. Based on the histology and stage determined post-nephrectomy, appropriate chemotherapy can be initiated tailored to the specific characteristics of the tumor. Explanation of other choices: A: Core biopsy followed by chemotherapy is not recommended as it may not provide sufficient tissue for histologic diagnosis and staging. C: Fine-needle aspiration is not adequate for a definitive diagnosis or staging and may not provide enough tissue for necessary testing. D: Neoadjuvant chemotherapy before surgery is not appropriate in this scenario as the primary tumor needs to be resected for accurate staging and histologic evaluation.
Question 3 of 5
A 7-year-old Hispanic male is referred to the hematology consult service by his pediatrician because of concern for hemoglobinopathy. In his records, you find a hemoglobin electrophoresis performed last year which shows hemoglobin A 78% and hemoglobin F 22%. His complete blood count is normal, and he has normal growth and development. Which of the following is true for this patient?
Correct Answer: D
Rationale: The correct answer is D because hereditary persistence of fetal hemoglobin (HPFH) is a condition where there is a lifelong elevation of fetal hemoglobin (HbF) levels. In HPFH, the distribution of HbF is pancellular, meaning it is present in all red blood cells. This patient's hemoglobin electrophoresis shows a significant amount of HbF (22%), which is consistent with HPFH. Choice A is incorrect because a significant elevation of HbF in a 7-year-old is not considered normal and warrants further investigation. Choice B is incorrect because delta-beta thalassemia typically presents with a different hemoglobin pattern on electrophoresis. Choice C is incorrect because delta-beta thalassemia can indeed cause microcytosis due to ineffective erythropoiesis.
Question 4 of 5
A newborn male has severe bleeding after circumcision, resulting in the need for a blood transfusion. You are called to consult on this child, and you diagnose him with severe hemophilia A. Upon taking a family history, you note that no other family members have hemophilia, other bleeding disorders, or a bleeding diathesis. Which of the following is the most likely outcome of genotyping the Factor VIII gene?
Correct Answer: C
Rationale: The correct answer is C: An inversion mutation in the F8 gene will be identified. In hemophilia A, around 50% of cases are due to large intragenic inversions involving the Factor VIII gene. In this scenario, since there is no family history of hemophilia or other bleeding disorders, the most likely genetic cause is an inversion mutation in the F8 gene. This type of mutation is not dependent on a family history and can occur spontaneously. Missense mutations (choice B) and nonsense mutations (choice D) are less common in hemophilia A and are not the typical genetic abnormalities associated with this condition. Choice A is incorrect because the absence of a family history does not rule out the possibility of a genetic mutation.
Question 5 of 5
A healthy 17-year-old African American male presents with a thrombosis of the right upper extremity. His past medical history is remarkable only for sickle cell trait. The history is negative for recent risk factors for thrombosis (illness, surgery, immobility). He is the pitcher for his high school baseball team. Imaging confirms anatomical compression/narrowing of the right subclavian vein. Which of the following interventions is most likely to decrease this patient's long-term risk of recurrent thrombosis?
Correct Answer: D
Rationale: The correct answer is D: Resection of right first rib. The patient likely has thoracic outlet syndrome (TOS), which is causing compression of the subclavian vein leading to thrombosis. Resection of the right first rib is the definitive treatment for TOS, as it relieves the compression on the vein. This intervention addresses the root cause of the thrombosis, reducing the risk of recurrence. A: Systemic thrombolysis is not indicated in this case as it carries an increased risk of bleeding and may not address the underlying cause of the thrombosis. B: Catheter-directed thrombolysis is not the primary treatment for TOS and may not prevent recurrence. C: Anticoagulation with LMWH does not address the underlying compression of the vein and is not the primary treatment for TOS.