PID informations

PID Information

Booklets on 6 primary immunodeficiencies

Thanks to a generous sponsorship from CSL Behring and great support from the Immune Deficiency Foundation (IDF) USA it has been possible for IPOPI to produce booklets with descriptions of 6 different immunodeficiencies:

• X-Linked agammaglobulinemia
• Chronic Granulomatous Disease
• Hyper IgM Syndrome
• Severe Combined Immunodeficiency
• Wiscott Aldrich Syndrome
• Common Variable Immunodeficiency.

This is the first set of information, which IPOPI hope to be able to expand to more languages and more descriptions of immunodeficiencies.

All these texts are based on information from the Immune Deficiency Foundation’s “Patient and Family Handbook on Primary Immunodeficiencies”, 4th edition.

National Member Organisations can request the booklets free of charge. Patients, nurses and doctors can obtain the booklets on request from:

Johan Prévot, Executive Director IPOPI www.ipopi.org

Firside, Main Road, Downderry, Cornwall PL11 3LE, UK

Portugal Office : Av. Aida, Bloco 8, Escritorio 821, 2765-187 Estoril, Portugal

Tel/Fax: +351 21 407 5720, Mobile: +351 91 228 5049

For larger deliveries a contribution to cover shipping expenses may be requested. The booklets have the same contents in English, French, German, Spanish and Portuguese and Russian. Click your preferred language to see the booklets:

List of Some Primary Immunodeficiencies

The World Health Organization (WHO) recognizes some 100 primary immunodeficiencies including X-Linked Agammaglobulinemia (Bruton's Disease), Common Variable Immunodeficiency (also called Hypogammaglobulinemia), Selective IgA Deficiency, and Severe Combined Immunodeficiency (boy-in-the-bubble disease).

Some disorders such as Selective IgA Deficiency can be quite common, occurring as often as 1/500 to 1/1000 individuals. Others may be as rare as 1 individual affected per million. Untreated primary immune deficiencies are characterized by frequent, some times life-threatening infections and debilitating illnesses.

Because of advances in medical understanding and treatment of primary immunodeficiency diseases, many patients who in the past would not have survived childhood are now able to live nearly normal lives. Most primary immunodeficient patients require life long therapies including intravenous gammaglobulin infusion, aggressive antibiotic therapies, or bone marrow transplantation.

Five classes of primary immunodeficiency diseases have been identified:

1. B-cell (B-lymphocyte) disorders (such as X-linked agammaglobulinemia, common variable immunodeficiency, and selective Immunoglobulin A deficiency);
2. Combined T- and B-lymphocyte disorders (such as severe combined immunodeficiency, i.e. SCID, the Wiskott-Aldrich syndrome and ataxia telangiectasia, the DiGeorge Syndrome and chronic mucocutaneous candidiasis),
3. Phagocytic disorders (such as chronic granulomatous disease) and
4. Complement disorders (such as C2 deficiency and C3 deficiency).
5. Diseases with recurrent fever and inflammatory manifestations.

Secondary immunodeficiencies result from environmental factors, some therapies etc. One example is the Acquired Immune Deficiency Syndrome (AIDS), which is caused by the HIV virus. Other immunodeficiency diseases occur or are acquired as the result of having cancer, severe nutritional disorders, burns, infections, exposure to radiation or organ transplantation.

This is a list of primary immunodeficiencies. It is by far not complete as WHO lists more than 100 different diagnoses.