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.

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.

Correct Answer: A

Rationale: The correct answer is A: Thrombosis. Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia and thrombocytopenia, not thrombosis. In TTP, there is excessive platelet aggregation leading to microthrombi formation in small blood vessels, causing hemolysis and thrombocytopenia. Neurologic deficits are also common due to microvascular ischemia. Thrombosis is not a typical feature of TTP and is more commonly associated with conditions like deep vein thrombosis or pulmonary embolism. Thrombosis is not a hallmark of TTP, making choice A the correct answer.

Correct Answer: A

Rationale: The correct answer is A: Immunophenotype - mature B cell. In acute lymphoblastic leukemia, a mature B cell immunophenotype is associated with a poor prognosis due to its resistance to treatment. Gender being female is a good prognostic factor as females tend to have better outcomes. Hyperploidy is favorable as it indicates a better response to treatment. Age 4 to 10 years old is also a good prognostic factor as younger age groups have better survival rates.

Correct Answer: A

Rationale: The correct answer is A: Nodular sclerosis. This subtype of Hodgkin's lymphoma is more common in young women and has a good prognosis due to its characteristic fibrotic bands dividing the lymph node into nodules. These nodules contain a mixture of inflammatory cells and Reed-Sternberg cells. Choice B: Mixed cellularity is more common in older patients and has a less favorable prognosis due to its heterogeneous cell population with abundant Reed-Sternberg cells. Choice C: Lymphocyte predominance typically affects young adults and has an indolent course with a good prognosis, but it is not as common in young women as nodular sclerosis. Choice D: Lymphocyte depletion is a rare subtype seen in older individuals with a poor prognosis due to the paucity of lymphocytes and an abundance of Reed-Sternberg cells.