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Pure Red Cell Aplasia (PRCA)

Pure Red Cell Aplasia (PRCA): Clinical Overview and Management

Pure Red Cell Aplasia (PRCA) is a rare hematologic disorder characterized by isolated normocytic anemia due to the bone marrow’s failure to produce red blood cell precursors (erythroblasts). White blood cells and platelets typically remain unaffected. Below is a comprehensive synthesis of its etiology, clinical features, and evidence-based management strategies.

Classification

PRCA is categorized into two primary forms:

Type	Characteristics
Acquired PRCA	Autoimmune or secondary to medications, infections, or thymomas. Common in adults.
Congenital PRCA	Diamond-Blackfan anemia (DBA), a genetic disorder diagnosed in infancy/childhood.

Causes and Risk Factors

PRCA arises from immune-mediated suppression of erythropoiesis or direct erythroid precursor damage:

1. Autoimmune mechanisms (most common):
   • Autoantibodies targeting erythroblasts or erythropoietin (EPO) receptors12.
   • Associated with thymomas (15–20% of cases), systemic lupus erythematosus (SLE), or rheumatoid arthritis13.
2. Infections:
   • Parvovirus B19 (targets erythroid P antigen in immunocompromised patients)14.
3. Medications:
   • Erythropoiesis-stimulating agents (ESAs), mycophenolate, phenytoin, or sulfonamides15.
4. Lymphoproliferative disorders:
   • Chronic lymphocytic leukemia (CLL) or large granular lymphocyte (LGL) leukemia36.

Symptoms

Symptoms reflect anemia severity and include:

• Fatigue, pallor, and dizziness15.
• Shortness of breath or tachycardia during exertion45.
• Headaches and pulsatile tinnitus in chronic cases5.

Diagnosis

Diagnostic criteria:

1. Complete blood count (CBC):
   • Normocytic anemia (Hb <12 g/dL in women, <13.5 g/dL in men) with reticulocytopenia (<10,000/μL)16.
2. Bone marrow biopsy:
   • Absent or reduced erythroid precursors (proerythroblasts), normal myeloid/megakaryocytic lineages13.
3. Additional testing:
   • Parvovirus B19 PCR (serum or marrow)1.
   • Chest CT to rule out thymoma13.
   • Autoantibody panels (anti-EPO antibodies in ESA-induced cases)56.

Treatment Strategies

First-Line Therapies

• Immunosuppression:
  • Corticosteroids (prednisone 1 mg/kg/day) induce remission in 30–60% of idiopathic cases25.
  • Cyclosporine (3–5 mg/kg/day) for steroid-refractory cases26.
• Thymectomy:
  • Indicated for thymoma-associated PRCA, achieving remission in ~30% of patients13.

Second-Line Therapies

• Monoclonal antibodies:
  • Rituximab (anti-CD20) or alemtuzumab (anti-CD52) for refractory/relapsed cases6.
• IV immunoglobulin (IVIG):
  • Effective for parvovirus B19-associated PRCA (0.4 g/kg/day for 5 days)14.

Supportive Care

• Blood transfusions for symptomatic anemia, with iron chelation if chronic5.
• Discontinue causative drugs (e.g., ESAs, mycophenolate)15.

Prognosis

• Acquired PRCA:
  • 80% achieve remission with immunosuppression, though relapses occur in 30–40%26.
  • Thymoma-associated PRCA has better outcomes post-resection3.
• Parvovirus-induced PRCA: Full recovery with IVIG in >90% of cases14.

Conclusion
PRCA is a treatable anemia with diverse etiologies requiring tailored immunosuppressive or supportive therapies. Early diagnosis and addressing underlying causes (e.g., thymoma, parvovirus) are critical for optimizing outcomes.

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At DrStemCellsThailand (DRSCT)‘s Anti-Aging and Regenerative Medicine Center of Thailand, we emphasize comprehensive evaluations and personalized treatment plans of Cellular Therapy and Stem Cells for managing various health conditions. If you have questions about Pure Red Cell Aplasia (PRCA) or would like more information on our services, consult with our experts today!

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References

1. MSD Manual: PRCA
2. Rare Diseases: PRCA
3. Wikipedia: PRCA
4. AAMDS: PRCA
5. Cleveland Clinic: PRCA
6. Blood Journal: PRCA