**14. Vaccination**

for the XLA patients who are unable to produce sufficient antibodies against antigens. IgG is purified from thousand of human plasma and contains a wide range of antibodies against so many infections. Thus, it is life saving for XLA patients, and they have to continue to receive to survive. The aim of immunoglobulin treatment given by intravenous (IVIG) or subcutane‐ ous (SCIG) infusions is to avoid acute infections, to decrease the number of bacterial infections,

IVIG infusions have to be done at hospital or home by professionally educated staff if possible. The common recommended dose of IVIG treatment for antibody replacement is between 0.3 and 0.6 g/kg, administered every 2 to 4 weeks via the intravenous route. The first IVIG infusion must be given slowly starting with a rate of 0.5 to 1.0 mg/kg per minute. Patient should be monitored closely for any adverse reactions during infusion. If the patient tolerates well, the infusion rate may be increased to 1.5 to 2.5 mg/kg per minute after 15 to 30 minutes. The maximal infusion rate is 4 mg/kg per minute, and infusion of an IVIG product should last 2 to 4 hours. The aim of IVIG therapy in patients with PID is to maintain serum IgG levels between 350 and 500 mg/ dL. Since there is a large variation in individual IgG elimination rates, the periodic measurement of serum IgG concentration is critical to monitor the adequacy of replacement during therapy. Retrospective studies in patients with XLA revealed that the severity and the number of infections especially pulmonary diseases are decreased depending on IVIG dose [88]. Serious bacterial illnesses and enteroviral meningoencephalitis were

A 5-year multicenter prospective study on 201 patients with CVID and 101 patients with XLA was conducted to identify the effects of long-term immunoglobulin treatment and the IgG trough level to be maintained over time required to minimize infection risk. Overall, 24% of patients with XLA remained infection free during the study. In addition, in XLA, the comor‐

Infusion-related adverse effects and transmission of blood-borne viruses are adverse effects of immunoglobulin replacement therapy [97]. Reduced adverse reactions are reported with improved and new IVIG products. The subcutaneous IG (SCIG) therapy was reported to be effective, safe, and well tolerated in children and adults. High treatment satisfaction (TS) scores and health-related quality of life (HRQOL) were advantages of SCIG. Subcutaneous infusions are recommended to patients who are small children, reactive to IVIG, or have problem with vascular access. SCIG is given as a parent-managed or a self-managed treatment. Norway, Sweden, United Kingdom, and Belgium are the countries in which SCIG is often applied to children [98, 89]. Clinical records of 1151 XLA patients identified from ESID were included in ESID registry. According to ESID registry, 305 XLA patients were treated with IVIG (73%) and

Bacterial infections treated with a high dose of selected antibiotics or antibiotics sensitive to

Six young patients with XLA treated with cord blood or bone marrow transplants were reported. No one benefited from transplantation, and expected increase in serum IgM or blood

bidity risk factor identified for pneumonia was the presence of bronchiectasis [96].

to improve quality of life, and to increase life expectancy of patients [8, 9, 47, 88–92].

prevented when maintained IgG levels were above 800 mg/dL [89–95].

114 patients were treated with SCIG (27%) [98, 99].

yielded pathogens for prolonged periods.

232 Immunopathology and Immunomodulation

B-cell number was not observed [100].

Live viral vaccines, such as oral polio, rotavirus, yellow fever, live attenuated influenza, and live bacterial (e.g., typhoid [*Salmonella typhi*, Ty21a]) vaccines should not be applied to patients with X-linked agammaglobulinemia. Therefore, inactivated polio vaccine (Salk) should be given to patients with XLA and their family contacts. These patients may develop vaccineacquired diseases such as central nervous system infection due to oral poliovirus vaccine.

The effectiveness of the measles and varicella vaccines are uncertain because most patients receive IVIG and do not have capacity to produce antibody responses [101, 102].

However, bacille Calmette–Guérin (BCG) vaccine applied to 50 patients with X-linked agammaglobulinemia did not reveal any systemic infection [102].
