Certified Pediatric Hematology Oncology Nurse (CPHON) Practice Exam

Congenital aplastic anemia is characterized by the failure of the bone marrow to produce adequate amounts of blood cells, leading to various complications such as anemia, increased risk of infections, and bleeding issues. Fanconi Anemia is the most common form of congenital aplastic anemia. It is a genetic disorder that not only results in aplastic anemia but also affects multiple organ systems and is linked to an increased risk of malignancies.

Fanconi Anemia arises from mutations in genes responsible for DNA repair, leading to bone marrow failure as a consequence of damage accumulating in hematopoietic stem cells. The clinical presentation often includes physical anomalies, varying degrees of cytopenias, and varying levels of end-organ dysfunction, making it a unique condition within the spectrum of aplastic anemias.

In contrast, Dyskeratosis Congenita primarily affects the skin and mucous membranes and is also associated with bone marrow dysfunction but is less frequently diagnosed solely as aplastic anemia. Thalassemia involves the production of abnormal hemoglobin rather than aplastic bone marrow function and presents with symptoms related to hemolytic anemia. Severe Combined Immunodeficiency (SCID) is primarily an immunological disorder rather than a condition of bone marrow failure. Thus, Fan