About pure red blood cell aplasia

What is pure red blood cell aplasia?

Acquired Pure Red Cell Aplasia is a rare bone marrow disorder characterized by an isolated decline of red blood cells (erythrocytes) produced by the bone marrow. Affected individuals may experience fatigue, lethargy, and/or abnormal paleness of the skin (pallor). Acquired Pure Red Cell Aplasia may occur for unknown reasons (idiopathic) or as a primary autoimmune disorder. It is also believed that Acquired Pure Red Cell Aplasia may occur secondary to a tumor of the thymus gland (thyoma), viral infections, or certain drugs.

What are the causes for pure red blood cell aplasia?

Pure red cell aplasia (PRCA) is a very rare blood production disorder that affects the precursor of the red blood cell which are called reticulocytes. In PRCA, the bone marrow stops producing red blood cells, and this leads to low red blood cell count or anemia. This disorder usually affects adults. Red blood cells are responsible for carrying oxygen to various cells and tissues.

What causes PRCA?
According to the most recent cases, PRCA is thought to be due to autoimmune diseases, but some other idiopathic reasons are also thought of (the ones whose cause can’t be found). Others include:
1. The Parvovirus B19 infection can also lead to damage in the reticulocytes (or the precursors).
2. Auto-immune disorders like Rheumatoid arthritis and Lupus, can also cause PRCA.
3. Thymoma (cancer in the thymus gland).
4. Drugs like Erythropoietin and mycophenolic acid.
5. Congenital- The term “hereditary pure red cell aplasia” has been used to refer to Diamond -fan anemia, which is a very rare kind of anemia and only several hundred cases are seen worldwide. This is a genetic condition usually diagnosed in the first 2 years of life. Its symptoms involve physical malformations and mental retardation.
6. Common variable immunodeficiency (CVID).

Symptoms
Fatigue,Dizziness,Pale skin,headaches,shortness of breath,Rapid heart rate
Conditions
Anemia
Drugs
NA

What are the treatments for pure red blood cell aplasia?

Pure red cell aplasia (PRCA) is a very rare blood production disorder that affects the precursor of the red blood cell, which are called reticulocytes. In PRCA, the bone marrow stops producing red blood cells, and this leads to low red blood cell count or anemia. This disorder usually affects adults. Red blood cells are responsible for carrying oxygen to various cells and tissues.

Treatment-
The aim of treatment is to restore red blood cell production while also treating any underlying disorder. The following are the most common treatments:
1. Blood transfusion- to increase the level of red blood cells, if the anemia is really severe and the patient is not responding to any other treatments. But blood transfusions may cause some allergic side effects.
2. Corticosteroids- like prednisone, is usually the starting treatment for PRCA. This method also includes severe side effects, so they are rarely used.
3. Immunosuppressive therapy- Immunosuppressive drugs may be tried if the cause of red cell aplasia is an immune system disease. Cyclosporine, cyclophosphamide, 6-mercaptopurine, azathioprine, and antithymocyte globulin are among them. Immunosuppressive therapy necessitates a brief hospitalization, and recurring courses may be required.
4. Immunoglobulins- If the parvovirus persists, slow transfusions of immune globulins into a vein every three or four weeks may be administered. Some side effects of these drugs include headache, dizziness, flushing, muscle cramps, joint pain, as well as reactions at the injection site (pain, swelling, redness). Kidney problems may occur in some cases.

Symptoms
Fatigue,Dizziness,Pale skin,headaches,shortness of breath,Rapid heart rate
Conditions
Anemia
Drugs
NA

What are the risk factors for pure red blood cell aplasia?

Pure red cell aplasia (PRCA) is a very rare blood production disorder that affects the precursor of the red blood cell, which are called reticulocytes. In PRCA, the bone marrow stops producing red blood cells, and this leads to low red blood cell count or anemia. This disorder usually affects adults. Red blood cells are responsible for carrying oxygen to various cells and tissues.

Risk factors of PRCA include-
1. Experiencing autoimmune diseases like rheumatoid arthritis, hepatitis, or a large population of clonal large granular lymphocytes that target red cell precursors (reticulocytes) in the bone marrow.
2. Tumors in the thymus gland are called thymomas.
3. Viruses such as parvovirus B19
4. Certain hereditary genetic disorders that manifest primarily in childhood

What causes PRCA?
According to the most recent cases, PRCA is thought to be due to autoimmune diseases, but some other idiopathic reasons are also thought of (the ones whose cause can’t be found). Others include:
1. The Parvovirus B19 infection can also lead to damage in the reticulocytes (or the precursors).
2. Auto-immune disorders, like Rheumatoid arthritis and Lupus, can also cause PRCA.
3. Thymoma (cancer in the thymus gland).
4. Drugs like Erythropoietin and mycophenolic acid.
5. Congenital- The term “hereditary pure red cell aplasia” has been used to refer to Diamond -fan anemia, which is a very rare kind of anemia, and only several hundred cases are seen worldwide. This is a genetic condition usually diagnosed in the first 2 years of life. Its symptoms involve physical malformations and mental retardation.
6. Common variable immunodeficiency (CVID).

Symptoms
Fatigue,Dizziness,Pale skin,headaches,shortness of breath,Rapid heart rate
Conditions
Anemia
Drugs
NA

Is there a cure/medications for pure red blood cell aplasia?

Pure red blood cell aplasia is a rare condition concerned with blood production in the bone marrow. This spongy tissue located in the center of the bones fails to work in its appropriate manner, which further results in anemia.

List of drugs
Corticosteroids

1. Prednisones like Delta-Cortef and Econopred
2. Cyclophosphamides like Cytoxan and Neosar
3. 6-Mercaptopurine like Purinethol
4. Azathioprine like Imuran
5. Cyclosporine like Sandimmune and Neoral
6. Antithymocyte globulin like Thymoglobulin
7. Intravenous immune globulin like Gamimune, Gammagard, Sandoglobulin, Gammar-P
8. Danazol like Danocrine

Treatment
1. Immunosuppressive therapy: If red cell aplasia originates in an immune system disorder, then immunosuppressive drugs can be tried. These involve cyclosporine, cyclophosphamide, 6-mercaptopurine, azathioprine and anti-thymocyte globulin. This treatment seeks a brief period of hospitalization, and repetition of courses may be required.

2. Immunoglobulins: Gradual infusions of intravenous immunoglobulins every three to four weeks may be scheduled if there is a persistent parvovirus.

3. Blood transfusion: If anemia is extremely severe and unresponsive to various treatments, or if unbearable side effects are experienced, blood transfusion is a good option. They are usually set every four to six weeks.

4. Chelation therapy: Chelation therapy is the surgical extraction of iron from the body in the case of an overload of iron through chronic blood transfusions. Deferoxamine is given in an injection for eight to twelve hours, five to seven nights weekly.

5. Removal of the thymus: The thymus is removed if a tumor is found in the thymus.

Symptoms
Fatigue,Dizziness,Pale skin,headaches,shortness of breath,Rapid heart rate
Conditions
Anemia
Drugs
NA

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