What is Pure Red Cell Aplasia?
Pure Red Cell Aplasia or erythroblastopenia is a form of anaemia, a blood disorder caused by a reduction in the number of red blood cells.
There are many different forms of anaemia. But people with Pure Red Cell Aplasia have bone marrow which is incapable of producing red blood cells.
Symptoms of the disease are the same for all types of anaemia:
- pallor
- fatigue
- elevated heart-rate
- dizziness
- shortness of breath
- insomnia
- difficulty concentrating
Because Pure Red Cell Aplasia comes on gradually someone with the disease might not outwardly appear to be as ill as their acute level of anaemia would suggest.
The disease has three types:
Acquired Pure Red Cell Aplasia — a rare condition, characterised by an absence of immature red blood cells (reticulocytes) in the bone marrow and a low red blood cell count.
Acute Pure Red Cell Aplasia — the most common type. It is identical to the acquired form, but it is usually triggered by a virus or a drug and spontaneously goes away.
Inherited Pure Red Cell Aplasia — the congenital form is also called Diamond-Blackfan Anaemia. It is extremely rare, is usually diagnosed within the first two years of life and can include physical abnormalities.
Diagnosis is made via a blood count and a bone marrow biopsy. The disease very rarely affects children.
How is it treated?
The disease can be, but is not always, related to another condition, such as the cancer thymoma, some forms of leukaemia and viruses such as HIV, hepatitis and herpes.
Treatment for Pure Red Cell Aplasia may also be targeted at underlying disorders that could be causing it.
Blood transfusions can be administered to those with the disease in order to maintain a healthy red blood count. However, because this carries a risk of iron overload, it must be accompanied by chelation therapy to remove iron from the bloodstream.
Steroids can be used in the short-term to stimulate the production of red blood cells by the bone marrow. But this does not work in all cases and an unwanted side effect of extended usage is osteoporosis.
Immunosuppressive drugs can be used in some cases. Cyclosporine A is one of the leading drugs of this type. However, remission rates are high with this method of treatment.
The majority of cases of Acute Pure Red Cell Aplasia are caused by a parvovirus B19 infection. In these cases, you can be treated with immunoglobulin infusions. The prognosis for the transient Acute Pure Red Cell Aplasia is excellent.
How treatable the other forms are depends on the underlying condition with which it is associated.
Pure Red Cell Aplasia can result in Aplastic Anaemia and Acute Myelogenous Leukaemia — which is why it is important to treat it early.
