title: Primary vs. secondary antibody deficiency: clinical features and infection outcomes of immunoglobulin replacement.
abstract: Secondary antibody deficiency can occur as a result of haematological malignancies or certain medications, but not much is known about the clinical and immunological features of this group of patients as a whole. Here we describe a cohort of 167 patients with primary or secondary antibody deficiencies on immunoglobulin (Ig)-replacement treatment. The demographics, causes of immunodeficiency, diagnostic delay, clinical and laboratory features, and infection frequency were analysed retrospectively. Chemotherapy for B cell lymphoma and the use of Rituximab, corticosteroids or immunosuppressive medications were the most common causes of secondary antibody deficiency in this cohort. There was no difference in diagnostic delay or bronchiectasis between primary and secondary antibody deficiency patients, and both groups experienced disorders associated with immune dysregulation. Secondary antibody deficiency patients had similar baseline levels of serum IgG, but higher IgM and IgA, and a higher frequency of switched memory B cells than primary antibody deficiency patients. Serious and non-serious infections before and after Ig-replacement were also compared in both groups. Although secondary antibody deficiency patients had more serious infections before initiation of Ig-replacement, treatment resulted in a significant reduction of serious and non-serious infections in both primary and secondary antibody deficiency patients. Patients with secondary antibody deficiency experience similar delays in diagnosis as primary antibody deficiency patients and can also benefit from immunoglobulin-replacement treatment.
authors: Immunology Department, Barts Health NHS Trust, London, United Kingdom.
authors: Sai S
authors: SS
authors: Duraisingham
authors: Immunology Department, Barts Health NHS Trust, London, United Kingdom.
authors: Matthew
authors: M
authors: Buckland
authors: Immunology Department, Barts Health NHS Trust, London, United Kingdom.
authors: John
authors: J
authors: Dempster
authors: Immunology Department, Barts Health NHS Trust, London, United Kingdom.
authors: Lorena
authors: L
authors: Lorenzo
authors: Immunology Department, Barts Health NHS Trust, London, United Kingdom.
authors: Sofia
authors: S
authors: Grigoriadou
authors: Immunology Department, Barts Health NHS Trust, London, United Kingdom.
authors: Hilary J
authors: HJ
authors: Longhurst
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications / Q000175:diagnosis* / Q000209:etiology*; D000368:Aged; D000369:Aged, 80 and over; D001987:Bronchiectasis / Q000175:diagnosis / Q000209:etiology; D015331:Cohort Studies; D057210:Delayed Diagnosis; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000009:adverse effects; D007239:Infections / Q000209:etiology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D063868:Patient Outcome Assessment; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007136:Immunoglobulins; D007166:Immunosuppressive Agents