📝 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

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Red cell aplasia