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RN Nursing · Pathophysiology · Practice question

A young client has a genetic disorder of the red blood cells (RBC), where the RBCs and hemoglobin synthesis are abnormal. Based on this information, which type of anemia will the nurse educate the client and parents about?

Answer & explanation

Correct: Sickle cell anemia.

Sickle cell anemia is a genetic (inherited) disorder in which a mutation in the beta-globin gene produces abnormal hemoglobin S. When hemoglobin S is deoxygenated, red blood cells distort into a rigid, sickle shape. This matches the stem's description of a genetic disorder in which both the red blood cells and hemoglobin synthesis are abnormal. Iron-deficiency anemia results from inadequate iron stores leading to reduced hemoglobin production — it is nutritional or chronic-blood-loss in origin, not a genetic defect in RBC structure. Pernicious anemia is caused by vitamin B12 deficiency due to lack of intrinsic factor; again, it is not a genetic RBC structural defect. Aplastic anemia results from bone marrow failure leading to pancytopenia; while it can have genetic contributions in some forms, it is characterized by an absence of all blood cell production rather than abnormal hemoglobin synthesis. The combination of genetic origin, abnormal RBCs, and abnormal hemoglobin synthesis points specifically to sickle cell anemia as the correct answer for client and family education.

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