Acquired pure red cell aplasia

Acquired Pure Red Cell Aplasia

Acquired Pure Red Cell Aplasia (PRCA) is a rare hematological disorder characterized by a severe reduction in the production of red blood cells (RBCs) due to the selective destruction or suppression of erythroid progenitor cells in the bone marrow. This condition results in anemia, while the production of white blood cells and platelets remains normal.

Pathophysiology

PRCA is primarily caused by an immune-mediated attack on erythroid progenitor cells. The immune system mistakenly targets these cells, leading to their destruction or inhibition. This can be due to:

• Autoimmune disorders: Conditions such as systemic lupus erythematosus or rheumatoid arthritis can trigger PRCA.
• Thymoma: A tumor of the thymus gland is associated with PRCA in some patients.
• Viral infections: Infections such as parvovirus B19 can lead to transient PRCA.
• Medications: Certain drugs, including some antibiotics and immunosuppressants, have been implicated in the development of PRCA.

Clinical Presentation

Patients with PRCA typically present with symptoms of anemia, which may include:

• Fatigue
• Pallor
• Shortness of breath
• Tachycardia

Unlike other forms of anemia, PRCA does not usually present with jaundice or splenomegaly, as the destruction of red blood cells is not a feature of this condition.

Diagnosis

The diagnosis of PRCA is based on:

• Complete blood count (CBC): Reveals normocytic or macrocytic anemia with reticulocytopenia.
• Bone marrow biopsy: Shows a marked reduction or absence of erythroid precursors, with normal myeloid and megakaryocytic lineages.
• Serological tests: May be used to identify underlying autoimmune disorders or viral infections.

Treatment

The treatment of PRCA depends on the underlying cause:

• Immunosuppressive therapy: Corticosteroids, cyclosporine, or other immunosuppressants are commonly used.
• Treatment of underlying conditions: Removal of a thymoma or treatment of an associated autoimmune disorder.
• Intravenous immunoglobulin (IVIG): May be effective in cases associated with viral infections.
• Blood transfusions: Used to manage severe anemia until other treatments take effect.

Prognosis

The prognosis of PRCA varies depending on the underlying cause and response to treatment. Some patients achieve complete remission, while others may require long-term management.

Also see

• Anemia
• Bone marrow failure
• Thymoma
• Autoimmune diseases