ATI RN
Hematological System Questions
Question 1 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 2 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.
Question 3 of 5
Iron-refractory iron deficiency anemia (IRIDA) is a rare inherited condition characterized by congenital iron deficiency anemia, poor response to oral iron, and partial but incomplete response to intravenous iron therapy. Which is the genetic mutation associated with IRIDA?
Correct Answer: C
Rationale: The correct answer is C: TMPRSS6. Mutations in TMPRSS6 gene are associated with IRIDA. TMPRSS6 encodes matriptase-2, a key negative regulator of hepcidin, a hormone that controls iron absorption. Mutations in TMPRSS6 lead to impaired hepcidin regulation, resulting in reduced iron absorption and subsequent iron deficiency anemia. Choice A: TFR2 mutations are associated with hereditary hemochromatosis, not IRIDA. Choice B: H63D mutation in HFE gene is also associated with hereditary hemochromatosis, not IRIDA. Choice D: EPOR mutations are associated with congenital erythrocytosis, not IRIDA.
Question 4 of 5
The nurse is assessing an older client for any potential hematologic health problem. Which assessment finding is the most significant and would be reported to the primary health care provider?
Correct Answer: B
Rationale: The correct answer is B - Multiple petechiae and large bruises. This finding indicates potential bleeding issues or clotting disorders, which are serious hematologic health problems. Petechiae and bruises may suggest thrombocytopenia or other underlying conditions. Reporting this to the primary health care provider is crucial for further evaluation and management. A: Poor skin turgor on both forearms - Indicates dehydration, not necessarily a hematologic issue. C: Dry, flaky skin on arms and legs - Suggests a skin condition, not directly related to hematologic problems. D: Decreased body hair distribution - More indicative of endocrine or nutritional issues, not specific to hematologic health problems.
Question 5 of 5
A client is having a bone marrow aspiration and biopsy and is extremely anxious. What action by the nurse is the most appropriate?
Correct Answer: A
Rationale: The correct answer is A: Assess the client's fears and coping mechanisms. This is the most appropriate action because it allows the nurse to understand the client's specific anxieties and provide personalized support. By assessing the client's fears and coping mechanisms, the nurse can address any concerns and implement tailored strategies to help the client feel more comfortable. Choice B is incorrect because simply reassuring the client may not be effective in alleviating their anxiety. Choice C is incorrect as sedating the client should only be considered if other interventions are ineffective. Choice D is incorrect because bone marrow aspiration and biopsy are typically done with local anesthesia, not general anesthesia, so telling the client they will be asleep would be misleading.