Autoimmune neutropenia

Evidence: Unconfirmed

Names

Autoimmune neutropenia (AIN)
Autoimmune neutropenia of infancy
Primary autoimmune neutropenia

Description

Neutropenia is defined as a low white blood cell count. There are many forms of neutropenia connected to autoimmune disease. It is important to understand which form is which.

Primary neutropenia is diagnosed when there are no other conditions diagnosed (such as lupus, rheumatoid arthritis, or inflammatory bowel disease). Secondary neutropenia is a low white blood cell count that occurs when other diseases are present. This article only covers primary neutropenia.

Primary neutropenia most often appears in infants and children. The cause may be unrelated to autoimmune disease. Most cases of childhood primary neutropenia that are autoimmune are not serious and the disease goes away in 3 to 5 years with no long term effects. However, in a relatively small number of patients, the disease becomes chronic. Adults, mostly women, can also develop primary autoimmune neutropenia after childhood. These cases are almost always chronic.

In chronic autoimmune neutropenia the body produces IgG antibodies that target and destroy the body’s own white blood cells (“neutrophils”), which leads to a lowered white blood cell count and leaves patients with weakened immune systems susceptible to bacterial and other infections.

Patient Groups

National Neutropenia Network

Bloggers

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