Bruton Agammaglobulinemia Medication

Updated: Apr 22, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications.

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Immunoglobulins

Class Summary

Immunoglobulins are the mainstay of therapy. Passively supply a broad spectrum of IgG antibodies against bacterial, viral, parasitic, and mycoplasmic antigens. Check IgG levels every 3 months and then every 6 months when stable. The goal is to maintain IgG trough levels greater than 500 mg/dL in serum. Check liver function and kidney function 3-4 times a year.

Immune globulin intravenous (Gamimune, Gammar-P, Sandoglobulin, Gammagard)

Immune globulin intravenous neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; and may increase CSF IgG (10%).

Adjust the dose and interval according to individual needs.

Symptomatic adverse effects may be alleviated by premedicating with acetaminophen, diphenhydramine, or methylprednisolone (Solu-Medrol).

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Antibiotics

Class Summary

These agents treat common sinopulmonary infections (eg, pneumonia, otitis media). Drugs, such as amoxicillin and amoxicillin/clavulanate, are typical agents used. Fluoroquinolone therapy is useful for respiratory staphylococcal infections and for patients with allergies to other medications. If the infection is caused by Mycoplasma organisms, the drug of choice is clarithromycin. Severe infections may require hospitalization and IV therapy with ceftriaxone or vancomycin.

Amoxicillin (Amoxil, Trimox, Biomox)

Amoxicillin interferes with the synthesis of cell wall mucopeptides during active multiplication, resulting in bactericidal activity against susceptible bacteria.

Ceftriaxone (Rocephin)

Ceftriaxone is a third-generation cephalosporin with broad-spectrum gram-negative activity; it has lower efficacy against gram-positive organisms and higher efficacy against resistant organisms. Ceftriaxone arrests bacterial growth by binding to one or more penicillin-binding proteins.

Vancomycin (Vancocin, Lyphocin, Vancoled)

Vancomycin is a potent antibiotic directed against gram-positive organisms and active against Enterococcus species. It is useful in the treatment of septicemia and skin structure infections. Ii indicated for patients who cannot take or in whom no response has occurred with penicillins and cephalosporins or for those who have infections with resistant staphylococci. For abdominal penetrating injuries, it is combined with an agent active against enteric flora and/or anaerobes.

To avoid toxicity, the current recommendation is to assay trough levels after the third dose, drawn 0.5 hours prior to next dosing. Use creatinine clearance to adjust the dose in patients with renal impairment.

Vancomycin is used in conjunction with gentamicin for prophylaxis in patients allergic to penicillin undergoing GI or GU tract procedures.

Clarithromycin (Biaxin)

Clarithromycin inhibits bacterial growth, possibly by blocking the dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.

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Bronchodilators

Class Summary

Bronchodilators are administered via an inhaler to reduce bronchoconstriction and inflammatory response in the lungs. Inhaled beta2-agonists, with or without steroid inhalation therapy, are the standard of care for pulmonary maintenance in XLA.

Albuterol (Proventil, Ventolin)

Albuterol is a beta-agonist for bronchospasm refractory to epinephrine. It relaxes bronchial smooth muscle by its action on beta2-receptors, with little effect on cardiac muscle contractility.

Salmeterol (Serevent)

By relaxing the smooth muscles of the bronchioles in conditions associated with bronchitis, emphysema, asthma, or bronchiectasis, salmeterol can relieve bronchospasms. The effect may also facilitate expectoration. Adverse effects are more likely to occur when administered at higher or more frequent doses than recommended.

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Beclomethasone, inhaled (Qvar)

Beclomethasone inhibits bronchoconstriction mechanisms, produces direct smooth muscle relaxation, and may decrease the number and activity of inflammatory cells, in turn, decreasing airway hyperresponsiveness.

Fluticasone inhaled (Flovent)

Fluticasone inhibits bronchoconstriction mechanisms, produces direct smooth muscle relaxation, and may decrease the number and activity of inflammatory cells, in turn, decreasing airway hyperresponsiveness.

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