Are you always tired? Pale? Learn about this rare blood disease: Pure Red Cell Aplasia (PRCA)

Do you feel constantly exhausted, dizzy, or have difficulty walking up stairs? Someone may have asked you, "Why are you so pale these days?" These are often symptoms of anemia. But today we are going to talk about a rare condition that causes anemia, which many people have not heard of, but we should be aware of. That is Pure Red Cell Aplasia , or PRCA for short.

Simply put, what is Pure Red Cell Aplasia (PRCA)?

Imagine that inside our large bones, the bone marrow, is like a big blood-making factory. This factory is where the three types of cells our body needs, red blood cells, white blood cells, and platelets, are made. The main job of red blood cells is to pick up oxygen that comes into our lungs when we breathe and distribute it to all the other cells throughout the body.

Now, in a condition called Pure Red Cell Aplasia (PRCA) , the production line of the factory that makes red blood cells suddenly stops working. The production of other types of cells, namely white blood cells and platelets, usually doesn't change much. When only the production of red blood cells stops, the number of red blood cells in the body gradually decreases. This is what we call anemia . When there are not enough cells to carry oxygen throughout the body, the symptoms mentioned earlier, such as fatigue and weakness, begin to appear.

What are the most common symptoms of PRCA?

PRCA is a condition that causes severe anemia, so the symptoms are more obvious than those seen with normal anemia.

Why does such a rare situation occur?

There is no single cause for PRCA. There are several factors that contribute to it. Sometimes it can also occur as a side effect of another underlying medical condition.

• Autoimmune Diseases: Simply put, this is when our body's defense system (immune system) mistakenly attacks our own body. Sometimes this attack can also target the cells in the bone marrow that make red blood cells. For example, PRCA can occur with diseases like `(Systemic Lupus Erythematosus - SLE)` and `(Rheumatoid Arthritis)`.
• Blood Cancer: There is a risk of developing PRCA with certain types of blood-related cancers, especially Chronic Lymphocytic Leukemia (CLL) and Large Granular Lymphocytic Leukemia (LGL).
• Cancerous Tumors: Thymoma is a cancerous tumor that develops in the thymus gland in the chest. This can also be a major cause of PRCA.
• Viral Infections: Parvovirus B19The virus that causes PRCA is the main cause. This virus directly attacks the stem cells in the bone marrow that are responsible for making red blood cells. It can also be caused by HIV, Epstein-Barr virus, and hepatitis viruses.
• Bacterial Infections: Severe bacterial infections, such as tuberculosis, can also rarely cause PRCA.
• Certain medications: PRCA can also occur as a side effect of certain medications.
• Pregnancy: Very rarely, some women may experience temporary PRCA during pregnancy. However, the condition usually resolves spontaneously after delivery.

How does a doctor diagnose this disease?

If you have the symptoms mentioned above, a doctor will first examine you and then recommend several tests to confirm the diagnosis.

• Complete Blood Count (CBC): This measures the number of red blood cells, white blood cells, and platelets in your blood. In PRCA, the number of red blood cells is very low.
• Reticulocyte Count: This measures the number of immature red blood cells that are newly formed in the bone marrow and added to the blood. In PRCA, this number is also very low.
• Peripheral Blood Smear: A drop of blood is taken and examined under a microscope to check the shape and nature of the red blood cells.
• Parvovirus B19 Test: This test is performed if there is suspicion of a viral infection.
• CT Scan: This test can be done if there is a suspicion of a tumor like ``Thymoma'' in the chest.
• Bone Marrow Aspiration/Biopsy: This is the most important test to confirm the disease. A small needle is used to remove a sample of bone marrow from a place such as the hip bone under anesthesia. In PRCA, this can confirm that the bone marrow is completely depleted of red blood cell-forming cells.

How is PRCA treated?

PRCA is treated by identifying the cause of the disease and addressing that cause.

There are several treatment methods:

• Immunosuppressants: If the cause of the disease is a problem with the functioning of the immune system, drugs such as `(Corticosteroids)` (e.g. Prednisone) and `(Cyclosporine)` are given to control it.
• Blood Transfusion:If the blood count is very low and the symptoms are severe, blood transfusions are given as a quick relief. But this is a temporary solution. Since continuous blood transfusions can cause iron to build up in the body, separate treatment (Chelation Therapy) is required to remove the excess iron.
• Surgery: If the cause of the disease is a thymus gland tumor (Thymoma), surgery is performed to remove the gland (Thymectomy).
• Stem Cell Transplant: In some genetic conditions, stem cells taken from a healthy person are transplanted to try to create a new healthy bone marrow system.
• Immunoglobulin Therapy: If PRCA is caused by Parvovirus B19 infection, this treatment controls the virus.

Let's also learn about the side effects of treatments.

Like all treatments, PRCA treatments can cause some side effects. But your doctor will explain all of this to you in advance.

Side effects of immunosuppressant drugs

• Acne formation
• Risk of developing diabetes
• High Blood Pressure
• Osteoporosis
• Weight gain
• Stomach upset and nausea

Side effects of blood transfusion

• Fever and allergies (itching)
• Risk of infection
• Iron overload

What is it like to live with PRCA and how do I take care of myself?

PRCA can sometimes be a chronic condition. The disease can either completely resolve after treatment or it can recur. Therefore, there are several things to consider when living with this condition.

• See your doctor regularly: Even if your symptoms disappear after treatment, be sure to go for checkups at the intervals prescribed by your doctor. This will help you detect any risk of the disease returning early.
• Maintenance doses: Some people may need to continue taking medication at a lower dose to prevent the disease from returning.
• Healthy lifestyle:
• A good diet: Ask your doctor about iron-rich foods and a nutritious diet.
• Drink enough water: Prevent the body from dehydrating.
• Exercise: Engage in safe exercise that is appropriate for you, as advised by your doctor.
• Protect yourself from infections:Wash your hands often. Be careful in crowded places. Talk to your doctor about any necessary vaccinations.
• Pay attention to your symptoms: If you notice any changes in your body or any new symptoms, inform your doctor.