Chronic mild neutropenia in adults: relation to IgG3 deficiency and infection susceptibility

Abstract

IgG3 deficiency is common amongst chronic mild neutropenia patients, particularly in those with autoimmune background, but contributes not significantly to infection susceptibility.

Low plasma levels of the protein pro‐LL‐37 as an early indication of severe disease in patients with chronic neutropenia

“…Patients with AIN, IN, CN and SCN were included in this study and the clinical parameters of patients diagnosed during childhood are summarised in Table I. Eight patients diagnosed in adulthood with AIN or IN were described elsewhere (Karlstrom et al , 2001). Control subjects included four healthy adults and three paediatric postacute lymphoblastic leukaemia (ALL) patients with normal white blood cell counts and bone marrow appearance.…”

Neutropenia in antibody‐deficient patients under IVIG replacement therapy

“…This fact revealed a high and unexpected frequency of neutropenia, when compared with other studies in the medical literature (3,25). In addition, we also observed neutropenic episodes in patients with AT, SAD, and IgGSD, an extremely rare finding for these diseases (17)(18)(19)(20). These unusual results are probably due to the routine evaluation in our follow-up protocol, as per which serial blood cell count analysis is performed.…”

“…Primary immunodeficiencies are associated with neutropenia mainly in patients with X-linked agammaglobulinemia (XLA) (2-6, 8-10), X-linked hyper-IgM syndrome (XHIGM) (11,12), and common variable immunodeficiency (CVID) (13)(14)(15)(16). In ataxia telangiectasia (AT), IgG subclass deficiency (IgGSD), and specific antibody deficiency (SAD), it was only in case reports (17)(18)(19)(20). Many studies associate the resolution of neutropenia in XLA patients during intravenous immunoglobulin (IVIG) replacement (4)(5)(6)8).…”

Acute and chronic neutropenias. What is new?

“…Amongst less well characterized factors are the effect of a simultaneously reduced granulocyte function [2] and monocytosis (often seen in severe chronic neutropenia), coexisting polymorphism or mutations for other determinants of host defence, e.g. immunoglobulin or IgG subclass deficiencies [3], lack of mannose‐binding lectin [4] and other pattern recognition systems [5], as well as age, malnutrition, etc. [6, 7].…”

“…Recurrent infections are rarely seen. The risk of contracting bacterial infections may depend on the severity of neutropenia, but appears sometimes independent [3]. Low levels of plasma soluble CD16 molecules can be a marker for this propensity [48].…”