X-linked agammaglobulinemia natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Natural History, Complications and Prognosis

It is not recommended and dangerous for XLA patients to receive live attenuated vaccines such as live polio, or the measles, mumps, rubella (MMR vaccine).[1] Special emphasis is given to avoiding the oral live attenuated SABIN-type polio vaccine that has been reported to cause polio to XLA patients. Furthermore, it is not known if active vaccines in general have any beneficial effect on XLA patients as they lack normal ability to maintain immune memory.

XLA patients are specifically susceptible to viruses of the Enterovirus family, and mostly to: polio virus, coxsackie virus (hand, foot, and mouth disease) and Echoviruses. These may cause severe central nervous system conditions as chronic encephalitis, meningitis and death. An experimental anti-viral agent, pleconaril, is active against picornaviruses. XLA patients, however, are apparently immune to the Epstein-Barr virus (EBV), as they lack B cells needed for the viral infection.

It is not known if XLA patients are able to generate an allergic reaction, as they lack functional IgE antibodies.

There is no special hazard for XLA patients in dealing with pets or outdoor activities.[1]

Unlike in other primary immunodeficiencies XLA patients are at no greater risk for developing autoimmune illnesses.

References

  1. 1.0 1.1

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