Malcolm Rivera
Pure red blood cell aplasia, or PRCA, is a rare blood disorder that occurs when the bone marrow fails to produce an adequate number of red blood cells, resulting in anemia. This condition can be congenital or acquired, with a range of potential causes, including viral infections, autoimmune diseases, and certain medications. The most widely studied congenital form of PRCA is Diamond-Blackfan syndrome, a genetic condition typically diagnosed in early childhood. Acquired PRCA, on the other hand, usually affects adults and can arise from various underlying medical conditions or as a reaction to certain drugs.
| Characteristics | Values |
|---|---|
| Name | Pure Red Cell Aplasia (PRCA) |
| Description | A rare blood disorder that arises when the bone marrow fails to produce the normal number of red blood cells |
| Symptoms | Fatigue, weakness, pallor, and shortness of breath |
| Causes | Autoimmune diseases, collagen disorders, blood cancer, cancerous tumors, viral infections, drugs, and congenital conditions |
| Diagnosis | Complete blood count, reticulocyte count, blood smear, and bone marrow examination |
| Treatment | Blood transfusions, corticosteroids, immunosuppressants, and continuous follow-up |
| Prognosis | Varies depending on the underlying cause and response to treatment |
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What You'll Learn
Pure Red Cell Aplasia (PRCA)
PRCA is characterised by a reduction or absence of red blood cell precursors in the bone marrow, specifically a decrease in reticulocytes, which are immature red blood cells. This leads to common anaemia symptoms such as fatigue, weakness, pallor, and shortness of breath. In some cases, PRCA may also cause difficulty breathing (dyspnea), pulsatile tinnitus, and skin that appears paler than usual.
The acquired form of PRCA is typically seen in adults and can arise from various causes, including autoimmune disorders such as systemic lupus erythematosus, rheumatoid arthritis, and inflammatory bowel disease. It can also be triggered by certain medications, viral infections like parvovirus B19, hepatitis, HIV, and Epstein-Barr virus, and bacterial infections such as Group C streptococcus, tuberculosis, and bacterial sepsis. Additionally, cancerous tumours, specifically thymoma (thymic carcinoma), have been linked to PRCA.
The treatment for PRCA aims to restore red blood cell production and address any underlying disorders. Immunosuppressive therapy, such as cyclosporine A with or without corticosteroids, is often used. Blood transfusions may also be necessary to increase red blood cell levels, especially for patients who do not respond to other treatments. While PRCA can be a challenging condition to manage, understanding its underlying causes and providing appropriate treatments can lead to improvement or resolution of the disorder.
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Transient or Acute Self-limited PRCA
Pure red cell aplasia (PRCA) is a rare disorder characterised by a normocytic normochromic anemia with severe reticulocytopenia and a marked reduction or absence of erythroid precursors from the bone marrow. Transient or acute self-limited PRCA is the most common type of PRCA. It is typically triggered by a virus or drug and disappears once the virus clears or the responsible drugs are eliminated from the patient's system.
PRCA is an autoimmune disease, where the patient's immune cells mistakenly attack their own blood-forming stem cells. It can be caused by underlying conditions such as rheumatoid arthritis, hepatitis, or a large population of clonal large granular lymphocytes. All patients with PRCA have anemia, and symptoms can include a marked reduction in red blood cells. Doctors use a variety of tests to diagnose PRCA, including a complete blood count, reticulocyte count, blood smear, and bone marrow examination.
The prognosis for transient or acute self-limited PRCA is generally excellent, and patients with normal bone marrow function may recover without even knowing they had the condition. Treatment for PRCA typically involves corticosteroids, immunosuppressive therapy, or drug therapies, depending on the underlying cause. In cases caused by viruses, particularly B19 parvovirus, immunoglobulin infusions are used. Thymoma-associated PRCA requires surgical removal of the thymoma. While treatments can be effective, there is a significant relapse rate, and patients may require repeated treatment.
In summary, transient or acute self-limited PRCA is the most common form of PRCA, an uncommon autoimmune disorder. It is typically triggered by viral infections or drugs and has an excellent prognosis, with most patients recovering fully. Treatment options are available and can be repeated if necessary, but there is a risk of relapse.
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Inherited or Congenital PRCA
Pure red blood cell aplasia (PRCA) is an autoimmune disease that can be congenital or inherited. Congenital PRCA, also known as Diamond-Blackfan Anaemia (DBA), is a rare genetic condition usually diagnosed within the first two years of life. It is characterised as a disease of ribosomal biogenesis, with the bone marrow ceasing to produce red blood cells.
The congenital form of PRCA was first described by Joseph in 1936 and later by Diamond and Blackfan in 1938. It is a rare disease, with an incidence of approximately 5 to 7 cases per 1 million live births. The disease affects both sexes equally.
The congenital form of PRCA is associated with defective ribosomal genes, and allogeneic stem cell transplants have been successful in treating patients with this form of the disease. Sporadic cases are the most common, followed by cases with autosomal dominant inheritance. Rare instances of autosomal recessive inheritance have also been reported.
The diagnosis of PRCA requires a complete blood count with a differential to examine the total number and type of blood cells in the blood. A reticulocyte count is also necessary to determine the levels of young red blood cells. A bone marrow examination, including aspiration and biopsy, is required to confirm the diagnosis.
The treatment goal for PRCA is to restore red blood cell production and address any underlying disorders. Blood transfusions are often used to increase red blood cell levels, and corticosteroids, such as prednisone, are typically the initial treatment. If the underlying cause is immunological, immunosuppressive therapy may be used, and drug therapies such as cyclophosphamide, cyclosporine, and azathioprine may be administered.
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Diagnosis and treatment
Pure red cell aplasia (PRCA) is a rare disorder characterised by a reduction or absence of red blood cell precursors in the bone marrow, resulting in severe anaemia. Due to its rarity and varied presentations, diagnosing PRCA can be challenging. However, healthcare providers can effectively diagnose this condition by systematically evaluating the patient's medical history, conducting appropriate tests, and ruling out other potential causes of anaemia.
A complete blood count with a differential is performed to assess the total number and type of blood cells in the blood. A reticulocyte count is also done to determine the levels of young red blood cells. A blood smear allows doctors to examine the blood under a microscope. Bone marrow examination, including aspiration and biopsy, is necessary to understand how the bone marrow is affected. The histological picture from the bone marrow examination will depend on the cause of PRCA. For instance, autoimmune PRCA is characterised by a complete or near absence of erythroblasts, while the presence of large proerythroblasts with vacuolated cytoplasm and pseudopodia may indicate a B19 parvovirus infection.
The treatment goal for PRCA is to restore red blood cell production and address any underlying disorders. Blood transfusions are often used to increase red blood cell levels, especially in patients who do not respond to other treatments. Corticosteroids, such as prednisone, are commonly used as an initial treatment, but due to the large doses required and their potential side effects, they are rarely administered alone. Immunosuppressive therapy may be employed if the underlying cause is immunological, and the patient does not respond well to corticosteroids. Drug therapies in this category include cyclophosphamide, cyclosporine, azathioprine, and anti-thymocyte globulin. Rituximab may also be effective in certain cases. PRCA caused by viruses, particularly the B19 parvovirus, can be treated with immunoglobulin infusions. If a thymoma is present, surgical removal is typically required.
It is important to note that PRCA can be a lifelong condition, and ongoing treatment may be necessary to prevent relapse or recurrence. Patients should consult experienced haematologists and rheumatologists, if applicable, to determine the most appropriate treatment strategies based on their specific circumstances.
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Possible causes
Pure red blood cell aplasia (PRCA) is a rare blood disorder characterised by the inability of the bone marrow to produce red blood cells, resulting in anaemia. PRCA can be congenital or acquired and may arise from various causes.
Congenital PRCA
Also known as Diamond-Blackfan anaemia or Blackfan-Diamond syndrome, this is a genetic condition usually diagnosed during the first two years of life. It can be inherited or passed on to children. In some cases, it is caused by parvovirus, which can be transmitted transplacentally, damaging the fetal liver's erythroid progenitors and leading to severe red cell aplasia.
Acquired PRCA
This form of PRCA usually affects adults and can be triggered by a variety of factors, including:
- Autoimmune disorders: Systemic lupus erythematosus, rheumatoid arthritis, and inflammatory bowel disease are examples of autoimmune diseases that may cause PRCA.
- Blood cancers: Chronic lymphocytic leukaemia (CLL) and large granular lymphocytic leukaemia (LGL) are two of the most common blood cancers linked to PRCA.
- Cancerous tumours: Thymoma (thymic carcinoma) is the most common cancerous tumour associated with PRCA.
- Viral infections: Parvovirus B19 infection is a common cause of PRCA, particularly in individuals with weak immune systems or other underlying conditions affecting their bone marrow.
- Drugs: Medications such as diphenylhydantoin and rifampin have been implicated in drug-induced PRCA.
- Other conditions: People with certain medical conditions, such as Diamond-Blackfan anaemia, an inherited form of anaemia, may develop acquired PRCA.
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Frequently asked questions
Pure Red Cell Aplasia (PRCA).
It is a rare blood disorder where the bone marrow doesn't produce the normal number of red blood cells, leading to anemia.
The symptoms of PRCA include fatigue, weakness, pallor, and shortness of breath.
PRCA can be inherited or developed due to certain medical conditions, autoimmune diseases, blood cancer, cancerous tumors, or viral infections.
