Clinical characteristics

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Most patients with XLA are healthy at birth and remain well for the first few months of life. Recurrent infections, particularly otitis and pneumonia, usually develop sometime during the second 6 months of life, after the loss of maternally acquired antibody. The majority of patients with XLA are evaluated for immunodeficiency before 3 years of age. However, 20-30% of patients present later; some of these patients have higher concentrations of serum immunoglobulins and/or more B cells than are typical for XLA. A helpful clinical clue to the diagnosis of XLA is unusually small peripheral lymph nodes and tonsils. Germinal follicles, which constitute a considerable proportion of the volume of lymph nodes, are largely made up of B cells. In the absence of B cells, lymph nodes are quite small. Except for signs of past infection, there are no other striking physical findings. By the time of diagnosis approximately 80% of patients have experienced recurrent upper and/or lower respiratory tract infections. Diarrhea and pyoderma are also frequently seen before diagnosis and initiation of therapy. Between

Table 1 Characteristics of Bruton's agammaglobulinemia

X-linked recessive (occurs only in males)

Onset in the first 3 years of life

Recurrent infections with encapsulated bacteria

Sinusitis, otitis, bronchitis and pneumonia

Increased susceptibility to mycoplasma, enterovirus and hepatitis C

Marked decrease in serum IgG, IgM and IgA Profoundly reduced numbers of B cells Pre-B cells present in the bone marrow Treatment with gamma globulin and antibiotics Mutations in the gene for Bruton's tyrosine kinase (Btk)

20 and 30% of patients have experienced sepsis, meningitis, arthritis or failure to thrive. Profound neutropenia is present at the time of diagnosis in about 25% of patients. Overwhelming bacterial sepsis with rapid demise is not unusual. The most frequent infecting organisms in patients with XLA, and in all other patients with hypogammaglobulinemia, are Haemophilus influenzae, Streptococcus pneumoniae and Staphylococcus aureus.

After diagnosis and initiation of gamma globulin replacement therapy, upper and lower respiratory tract infections may continue to be a problem, particularly if the patient has developed chronic pulmonary changes prior to diagnosis. Diarrhea due to Giar-dia is not uncommon in patients with XLA but is usually easily treated. Mycoplasma or ureoplasma infections can cause persistent arthritis, pneumonitis or urologic problems. Between 5 and 10% of patients with XLA have chronic enteroviral infections resulting in meningitis/encephalitis, arthritis or a dermatomyositis-like syndrome. These infections are often slowly progressive and fatal. Although echovirus is the most common organism, vaccine-associated polio and Coxsackie have also been isolated.

High-dose gamma globulin therapy is expected to decrease the incidence of chronic lung disease and enteroviral or mycoplasma infections in patients with XLA; however, it has been the source of hepatitis C infection in some patients. At the present time, the majority of adolescents with XLA have intermittent or chronic mild sinusitis or bronchitis but are otherwise completely well with normal growth and development.

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