Do you often feel tired and lethargic? Do you find it difficult to breathe when you walk a little faster or climb stairs? Maybe you also feel that your skin is paler than usual. These can be signs of a lack of blood in the body, which is what we medically call anemia. But sometimes the cause of this anemia can be a more complex and rare disease than we think. Today we are going to talk about one such rare, but very important blood-related disease. That is Pure Red Cell Aplasia, which we call PRCA for short.
Simply put, what is this PRCA?
Think of the bone marrow in our body as a blood-making factory. This factory produces the three types of cells our body needs: red blood cells, white blood cells, and platelets. The main function of red blood cells is to carry the oxygen that comes into our lungs when we breathe to all the other cells in our body. It's like a truck service that delivers oxygen.
Now, in the case of PRCA, the blood-making factory, that is, the bone marrow, greatly reduces or completely stops producing only red blood cells . The production of other cell types (white blood cells and platelets) occurs normally. So when the number of red blood cells that carry oxygen to the body decreases, the organs in our body do not receive the oxygen they need. The severe anemia that results from this is the main problem of PRCA.
What symptoms might someone with PRCA experience?
PRCA causes the same symptoms as severe anemia, but they may be more severe than normal anemia. It is important for you to be aware of these symptoms.
| Symptom | A simple explanation |
|---|---|
| Fatigue | Feeling lifeless and constantly tired, no matter how much sleep you get. |
| Difficulty breathing (Dyspnea) | Feeling out of breath even after doing a small task or walking a short distance. |
| Dizziness | Feeling dizzy, especially when getting up from a sitting position. |
| Pale skin | The skin, lips, and under the nails become more white than usual. |
| Frequent headaches | Frequent headaches are caused by a decrease in the amount of oxygen needed by the brain. |
| Hearing a ringing in the ears (Pulsatile tinnitus) | A whistling sound is heard in the ears in accordance with the rhythm of the heartbeat. |
| Chest pain (Angina) | Chest pain due to the lack of oxygen to the heart muscles. |
The most important thing is, if you have one or more of these symptoms, don't panic and think it's PRCA. These can also be seen in normal anemia. But if these symptoms persist, it's definitely best to see a doctor and get advice.
Why does this situation called PRCA occur?
PRCA is not a single disease. There are several possible causes. Sometimes it can also be a side effect of another medical condition.
1. Immune system problems (Autoimmune Diseases)
This is the main cause of PRCA. Simply put, our body's defense system, the immune system, mistakenly starts attacking the stem cells in our own bone marrow that make red blood cells. Examples of such conditions include:
- Lupus disease (Systemic Lupus Erythematosus)
- Rheumatoid Arthritis
- Inflammatory Bowel Disease
2. Cancer
Some types of cancer can also cause PRCA.
- Blood-related cancers: Chronic Lymphocytic Leukemia (CLL) and Large Granular Lymphocytic Leukemia (LGL) are the two types of blood cancers that are mainly associated with this.
- Tumors: Thymoma, a cancer of the thymus gland, is the main cancerous tumor associated with PRCA.
3. Viral and bacterial infections
Some infectious conditions can also cause PRCA, either temporarily or permanently.
- Viruses: Parvovirus B19 is the most well-known of these. This virus directly attacks the cells in the bone marrow that are about to become red blood cells. HIV, Epstein-Barr virus, and hepatitis viruses can also cause it.
- Bacteria: Conditions such as Group C Streptococcus, Tuberculosis, and Bacterial Sepsis can also cause this.
4. Some medications
PRCA can also occur as a side effect of certain medications. For example, Erythrocyte-Stimulating Agents (ESAs), which are drugs given to kidney patients to increase red blood cell production, can sometimes cause this condition.
5. Pregnancy
Very rarely, some women may develop PRCA during pregnancy. Fortunately, the condition usually resolves on its own after the baby is born.
6. Genetic conditions that are present at birth
Diamond-Blackfan Anemia is a rare congenital anemia. It is usually diagnosed in childhood. These patients can also develop PRCA.
How do doctors accurately diagnose this disease?
When you go to a doctor with the symptoms mentioned earlier, he will examine you and, if he suspects this disease, refer you for several tests.
| Test | What do you see here? |
|---|---|
| Complete Blood Count (CBC) | The blood is checked to see if the red blood cell count is very low, and if the white blood cell and platelet counts are normal. |
| Reticulocyte Count | This refers to newly formed, immature red blood cells. In PRCA, this number is very low. This means that no new red cells are being formed. |
| Peripheral Blood Smear | A drop of blood is taken and examined under a microscope to examine the shape and nature of the red blood cells. |
| Bone Marrow Aspiration/Biopsy | This is the main test to confirm the disease . A very small sample of bone marrow is taken from a place such as the hip after anesthesia. In the case of PRCA, the stem cells that make red blood cells are not seen in it. |
| Other tests | To find the cause of the disease, tests such as Parvovirus B19 virus tests and CT scans (to check for lumps such as thymoma) can be done. |
What are the treatments for PRCA?
The treatment for PRCA depends on the cause of the disease. This means that not everyone is given the same treatment. The doctor will carefully study your condition and choose the most appropriate treatment.
- Immunosuppressants: If the cause of the disease is a problem with the immune system, these medications work to control the overactive immune system. Corticosteroids like Prednisone and Cyclosporine are commonly used for this.
- Blood Transfusion: If the anemia is very severe, blood is given externally to restore the blood level quickly. However, continuous blood transfusions can cause the body's iron levels to increase unnecessarily. In that case, separate treatment (chelation therapy) will be required to remove the excess iron.
- Surgery: If the cause of the disease is a thymoma, the treatment is surgical removal of the gland (Thymectomy).
- Immunoglobulin Therapy: If PRCA is caused by a parvovirus B19 infection, this treatment fights the virus and stops it from damaging red blood cells.
- Stem Cell Transplant: People with congenital conditions like Diamond-Blackfan Anemia can undergo a treatment in which healthy stem cells are transplanted to replace the diseased bone marrow.
Are there any side effects of the treatment?
Yes, like any treatment, there may be side effects. But there is no need to worry about them. Your doctor will inform you about all of these before starting treatment. He will also give you the necessary advice to manage the side effects.
For example, immunosuppressant drugs can increase your risk of infection, increase your blood pressure, and increase your weight. Blood transfusions can cause fever and allergies. Therefore, it is very important to pay close attention to changes in your body during treatment.
What do you need to know when living with PRCA?
PRCA can sometimes be a chronic illness. This means that even after treatment, the disease can sometimes recur. Therefore, it is essential to remain under medical supervision.
- Maintenance Therapy: Some people may need to continue taking immunosuppressant medications at a low dose to prevent the disease from returning.
- Continuous Follow-up: It is essential to see your doctor at regular intervals and have your blood tested. This can help you identify early on whether the disease will recur or develop other complications.
- Healthy lifestyle:
- Nutritious diet: It is important to eat a balanced diet, including iron-rich foods (spinach, meat, peas, lentils). Ask your doctor about the diet that is right for you.
- Drink enough water: Prevent the body from dehydrating.
- Protect yourself from infections: Wash your hands often. Be careful in crowded places. Get the necessary vaccinations.
- Exercise: Exercise at a level that your body can tolerate. Also, consult your doctor about this.
When you hear about PRCA, you may feel scared. That's normal. But remember, it's a treatable condition. The most important thing is to seek medical advice quickly and follow the treatment exactly, without ignoring the symptoms.
Take-Home Message
- PRCA is a rare blood disorder that stops the bone marrow from producing red blood cells.
- Its main symptoms are constant fatigue, paleness, and difficulty breathing.
- This can be caused by immune system problems, cancer, infections, and some medications.
- The main test to confirm the disease is a bone marrow biopsy.
- Treatment methods vary depending on the cause of the disease, and treatment can control symptoms.
- Because this can sometimes recur, long-term medical monitoring and regular checkups are very important.
- If you continue to have symptoms similar to anemia, don't panic and see your doctor immediately.
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