Management of Humoral Primary Immunodeficiencies in Adults

  • Gianfranco Vitiello
  • Giacomo EmmiEmail author
  • Boaz Palterer
Part of the Rare Diseases of the Immune System book series (RDIS)


Immunoglobulin replacement therapy is the cornerstone for the management of humoral primary immunodeficiencies as its major goal is the minimization of infective complications. Clinical phenotype and the presence of complications should guide the dosage of immunoglobulin replacement therapy and the choice between intravenous (IVIg) and subcutaneous (SCIg) route of administration. Mild infusion reactions are common with IVIg, whereas local pain, swelling, and erythema represent the major complaints with SCIg. Severe adverse reactions are rare and mainly associated to high-dose IVIg and comprise anaphylaxis, aseptic meningitis, and thrombosis.

Antibiotic prophylaxis is sometimes used in these patients, but standard protocols are still lacking. As unusual microorganisms and antibiotic resistance are common, an antibiogram should be obtained before initiating an empiric therapy.


Immunoglobulin replacement therapy Intravenous immunoglobulin Subcutaneous immunoglobulin Antibiotic prophylaxis Hematopoietic stem cell transplantation 


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Gianfranco Vitiello
    • 1
  • Giacomo Emmi
    • 1
    Email author
  • Boaz Palterer
    • 1
  1. 1.Department of Experimental and Clinical MedicineUniversity of FlorenceFlorenceItaly

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