Makindo Medical Notes

📝 Aide Memoire: Pure Red Cell Aplasia (PRCA) is a rare condition affecting a very small group of patients treated with Erythropoietin (EPO) or similar products.

📖 About Red Cell Aplasia

Red Cell Aplasia 🩸 is a group of disorders with selective absence or severe reduction of erythroblasts in the bone marrow 🦴, leading to reduced red blood cell (RBC) production. It may be congenital or acquired, and is distinct from aplastic anaemia which affects all lineages.

• 🧬 Rare haematological disorder
• ⬇️ Severe reduction in erythroblasts
• ❌ Leads to profound anaemia and related symptoms

🔎 Aetiology

• 🛡️ Immune-mediated destruction of erythroid progenitors
• 🦠 Infections (notably Parvovirus B19)
• 💉 Medications/treatments e.g. Erythropoietin (EPO)
• 🎭 Associated with haematological malignancies (e.g. lymphoma)
• 👶 Congenital e.g. Diamond-Blackfan Anaemia

📌 Causes

• 👶 Transient erythrocytopenia of childhood
• 🧬 Congenital: Diamond-Blackfan Anaemia
• 🦠 Parvovirus B19 infection: targets erythroid precursors
• 💉 EPO treatment: rare immune-mediated PRCA
• 🎭 Lymphoma: antibody-mediated suppression

🩺 Clinical Features

• 😴 Fatigue, weakness
• 😶 Pallor
• 😮‍💨 Dyspnoea on exertion
• 💫 Dizziness/lightheadedness
• ❤️ Palpitations/tachycardia (compensatory)

⚖️ Differential Diagnoses

• 🩸 Iron deficiency anaemia
• 🥦 Vitamin B12/folate deficiency
• 🦴 Aplastic anaemia (all lineages)
• 🧨 Haemolytic anaemia
• 🩺 CKD (EPO deficiency)
• 🔬 Myelodysplastic syndromes

🧪 Investigations

• FBC: Normocytic, normochromic anaemia + very low reticulocytes
• Serum EPO: Often elevated
• Bone marrow: 🔍 Few/no erythroid precursors
• Immunology: Anti-EPO antibodies (if EPO-related)
• Viral tests: Parvovirus B19 serology
• Genetics: For congenital forms (e.g. DBA)

💊 Management

• Supportive: 💉 Blood transfusions, supplements (iron, folate, B12)
• Immune-mediated: 💊 Corticosteroids, ciclosporin
• Infectious: 🦠 IVIG for Parvovirus B19
• Refractory: 🔄 Plasmapheresis (removes anti-EPO Abs)
• Secondary to malignancy: 🎭 Treat underlying lymphoma/cancer
• Severe/congenital: 🦴 Bone marrow transplant

📈 Prognosis

• Primary (immune-mediated): 👍 Good response to immunosuppression
• Secondary (infection/malignancy): Depends on underlying cause
• Congenital (DBA): Variable; may need long-term steroids or transplant
• Overall: ✅ Many patients achieve remission with appropriate therapy

📝 Conclusion

Red cell aplasia is a rare but significant disorder causing severe anaemia due to absence of erythroid precursors. Accurate diagnosis (FBC, marrow, viral & immunology testing) + tailored management (supportive, immunosuppressive, antiviral, or transplant) are essential. Early recognition 🕰️ prevents complications and improves survival.

📚 References

• Mayo Clinic: Pure Red Cell Aplasia
• NHLBI: Red Blood Cell Disorders
• WHO: Pure Red Cell Aplasia
• Arber DA et al. (2019). Diagnosis & Classification of Myeloid Neoplasms. Blood.
• Kim YB et al. (2020). Pure Red Cell Aplasia: An Update. Hem Oncol Clin N Am.
• American Society of Hematology: ASH PRCA resources