Certified Pediatric Hematology Oncology Nurse (CPHON) 2025 – 400 Free Practice Questions to Pass the Exam

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What is the typical treatment requirement for most patients with Diamond Blackfan Anemia?

Short course of steroids

Long-term steroids

The typical treatment requirement for most patients with Diamond Blackfan Anemia (DBA) involves long-term steroids. DBA is a rare inherited blood disorder characterized by a failure of the bone marrow to produce red blood cells, leading to anemia. Corticosteroids, like prednisone, are often effective in increasing red blood cell production in these patients, improving their hemoglobin levels and reducing the need for transfusions.

In many cases, the use of long-term steroids can help sustain red blood cell counts and manage symptoms associated with anemia, making it a primary approach in the management of DBA. While there are other treatment options available, such as blood transfusions for immediate symptom relief and bone marrow transplantation as a more definitive, yet risky, option, long-term steroid therapy remains one of the first-line treatments for many patients.

Short courses of steroids may be utilized in some contexts, but they generally do not suffice alone for managing the long-term symptoms of DBA. Blood transfusions are used primarily for more immediate management of severe anemia rather than as a long-term solution, and while bone marrow transplantation can offer a cure, it is typically reserved for cases that do not respond to medical management due to associated risks and logistical challenges. Thus, the long-term steroid approach

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Immediate blood transfusions

Bone marrow transplantation

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