What is acquired pure red cell aplasia?

What is acquired pure red cell aplasia?

Acquired pure red cell aplasia (PRCA) is a rare cause of profound anemia characterized by a very low reticulocyte count and the virtual absence of erythroid precursors in the bone marrow. All other cell lineages are present in normal numbers and appear morphologically normal.

How is pure red cell aplasia diagnosed?

A computed tomography (CT) scan of the chest may be done to look for a thymoma. A lymphocyte count in the blood will show whether or not you have a lymphocyte leukemia that may be causing pure red cell aplasia.

How is PRCA treated?

1 The most effective first-line treatment of idiopathic PRCA is cyclosporine A (CsA) administered at a starting dose of 2 to 6 mg/kg per day (in divided doses) possibly combined with steroid (prednisone at 30 mg/day) with a rapid taper, yielding an overall response rate (ORR) of about 65% to 87%.

How do you diagnose aplastic anemia?

Diagnosis

  1. Blood tests. Normally, red blood cell, white blood cell and platelet levels stay within certain ranges. In aplastic anemia all three of these blood cell levels are low.
  2. Bone marrow biopsy. A doctor uses a needle to remove a small sample of bone marrow from a large bone in your body, such as your hipbone.

Is pure red cell aplasia autoimmune?

Causes. Acquired Pure Red Cell Aplasia is thought to be an autoimmune disorder possibly caused either by a tumor of the thymus gland, certain drugs or a viral infection. It is one of a group of bone marrow failure syndromes.

How does pure red cell aplasia differ from aplastic anemia?

The major difference between PRCA and aplastic anemia is that, in PRCA, only the red blood cell line is affected, while the white blood cells and platelets remain at normal levels. In aplastic anemia, all three blood cell types are typically affected.

Is aplasia curable?

Treatment of Pure Red Blood Cell Aplasia Pure RBC aplasia secondary to parvovirus infection is treated with intravenous immune globulin. Thymectomy is done in patients with thymoma-associated pure RBC aplasia; most patients improve but are not always cured.

What is acquired sideroblastic anemia?

Sideroblastic anemia (SA) is a rare blood disorder that affects how your body produces red blood cells. If you have sideroblastic anemia, you have anemia from lack of red blood cells and too much iron in your system because your body’s not making use of iron in your red blood cells.

Does aplastic anemia show up on blood test?

The following tests can help diagnose aplastic anemia: Blood tests. Normally, red blood cell, white blood cell and platelet levels stay within certain ranges. In aplastic anemia all three of these blood cell levels are low.

What drugs can cause red cell aplasia?

Substances

  • Anticonvulsants.
  • Antitubercular Agents.
  • Immunosuppressive Agents.
  • Phenytoin.
  • Azathioprine. Isoniazid.

Is pure red cell aplasia curable?

If an individual with Acquired Pure Red Cell Aplasia has a tumor of the thymus gland, surgical removal of this gland often causes remission of this disorder.

Is pure red cell aplasia hereditary?

Patients with otherwise normal functioning bone marrow may even recover without having known they had it. Inherited or Congenital Pure Red Cell Aplasia ( Diamond-Blackfan Anemia ): Diamond-Blackfan anemia is a genetic condition usually diagnosed during the first two years of life.

What autoimmune disorders cause aplastic anemia?

Acquired Aplastic Anemia Other autoimmune diseases include rheumatoid arthritis and lupus. Acquired aplastic anemia can begin at any time in life. About 75 out of 100 cases of acquired aplastic anemia are idiopathic . This means they have no known cause.

What are the signs and symptoms of pure red cell aplasia?

Affected individuals may experience fatigue, lethargy, and/or abnormal paleness of the skin (pallor). Acquired Pure Red Cell Aplasia may occur for unknown reasons (idiopathic) or as a primary autoimmune disorder.

Acquired Pure Red Cell Aplasia is thought to be an autoimmune disorder possibly caused either by a tumor of the thymus gland, certain drugs or a viral infection. It is one of a group of bone marrow failure syndromes. Acquired Pure Red Cell Aplasia is a rare disorder affecting males and females in equal numbers.

When does red cell aplasia go into remission?

Acquired Pure Red Cell Aplasia usually goes into remission when certain drugs such as sulfonylureas (used for treating diabetes), gold for treatment of arthritis, penicillin, phenytoin and phenobarbitol used for treating epilepsy, or the anesthetic halothane which can cause this disorder are discontinued.

What is the best treatment for pure red cell aplasia?

Standard Therapies. Acquired Pure Red Cell Aplasia usually goes into remission when certain drugs such as sulfonylureas (used for treating diabetes), gold for treatment of arthritis, penicillin, phenytoin and phenobarbitol used for treating epilepsy, or the anesthetic halothane which can cause this disorder are discontinued.