Catholic University Catholic Hematology Hospital

Overview

Pure red cell aplasia (PRCA) is indeed a rare blood disorder where the bone marrow stops making red blood cells, which are needed to carry oxygen throughout the body. This selective decrease in red blood cells can lead to symptoms like fatigue, weakness, and paleness, but it doesn't affect the production of white blood cells (leukocytes), which fight infection, or platelets (thrombocytes), which help blood to clot. Because only the red blood cells are impacted, patients with PRCA don't usually have the increased risk of infection or bleeding that accompanies other types of bone marrow failure.

Causes and Symptoms

That's correct. While the exact genetic underpinnings of congenital pure red cell aplasia (PRCA) are not fully understood, there's evidence to suggest that genetics play a role in about one-fifth of the cases. Acquired PRCA, on the other hand, can develop after certain infections or as a reaction to specific medications. The symptoms of PRCA are similar to general anemia because the core issue is the lack of sufficient red blood cells. Affected individuals may experience headaches, dizziness, shortness of breath during physical activity (dyspnea on exertion), a noticeable paleness (pallor), and an overall feeling of tiredness (fatigue). These symptoms are the body's response to its cells not getting enough oxygen, which is the red blood cells' main job.

Treatment

The treatment plan for pure red cell aplasia (PRCA) is indeed tailored based on whether it is congenital or acquired: In congenital PRCA, steroids are often the initial treatment to help stimulate the bone marrow to produce red blood cells. The sooner steroid treatment is started, generally, the better the outcome. If steroids don't lead to improvement, then periodic red blood cell transfusions might be necessary to manage anemia, typically every one to two months.

For acquired PRCA, identifying and stopping any medications that might be causing the condition is a critical first step. Transfusions can help maintain adequate levels of hemoglobin and manage symptoms. Other treatment options, like splenectomy (removal of the spleen), androgens (which may help stimulate red blood cell production), and plasmapheresis (a process that filters the blood), are considered for more resistant cases or when first-line treatments fail to achieve the desired effect. These treatments are more invasive and are usually considered when other therapies have not been successful.