Patients with XLA will require lifelongreplacement therapies in order to replace immunoglobulin (Ig). Administering monthlyintravenous Ig (IVIg) or weekly subcutaneous immunoglobulins (SCIg) will resultin an improved clinical status with a decrease in serious infections such as pneumonia,meningitis and GI tract infections, studies have shown that administering equalamounts of IVIg and SCIg will yield a better immunological response .Long termIg replacement therapy will reduce the incidence and severity of infections aswell as prevent complications in organ function. Trough IgG levels shouldremain with normal limits (i.
e. 5-6 g/L) this is the minimum target level thatis recommended however studies show that these levels should be individualised (Jolles, et al., 2014) (Kumar & Clark,2005) . This method however is partially effective and is sometimes associatedwith long term complications. (Kerns, et al., 2010)Over the past few years the developmentof gene therapy through the transfer of hematopoietic stem cell transplantation(HSCT) could represent an alternative strategy for the treatment of Bruton’s disease.
This is done by replacing the patient’s hematopoietic stem cells (HSCs) thathave the pathogenic mutation with HSCs from a healthy donor. This is onlysuccessful if the stem cell donor is well matched, ideally this should be asibling or a close family member. Gene editing is another method that has beendeveloped that can correct the pathogenic causing gene whereby it could berepaired in its native chromosomal site or a new copy of the gene is directlyinserted into the impaired gene. This results in either the gene being turnedoff of being disrupted and so restore normal genetic function. Aggressive treatment such as higherdoses and longer courses of antibiotics maybe required in order to manage bacterialinfections experienced by the patient and thus prevent long term complications (Chin, Jyonouchi, Oleske, & Windle, 2014). Antibiotics such asamoxicillin and amoxicillin/ clavulanate can be used for sinopulmonary infections.
Ceftriaxone, Vancomycin and Cefotaxime can be used to eradicate streptococcuspneumococcus infections (Schwartz, et al., 2017).
