Pediatric Allergy and Immunology · 2008 · 13 citations · 21 references
Life SciencesHematologyImmunologyClinical ImmunologyImmunopathologyImmunotherapyImmune DysfunctionImmunodeficiency DisordersImmunoglobulin DisordersImmunosuppressionNeutrophil BiologyPrimary ImmunodeficiencyAntibody Deficiency SyndromesImmune-mediated NeutropeniaNeutropenia ProphylaxisNeutropenia Severity AssessmentNeutropenic Infections
Patients with antibody deficiencies are more prone to develop acute neutropenic episodes even during immunoglobulin replacement. The aims of this study were to evaluate the presence of acute neutropenia in 42 patients with primary antibody immunodeficiencies, currently receiving intravenous immunoglobulin (IVIG), and to describe the clinical and laboratory findings during neutropenic episodes. Of all patients, 10 (23.8%) presented acute neutropenia (absolute neutrophil count <1500 cells/mm3) during follow up (mean of 6.4 yr). The absolute neutrophil count ranged from 71 to 1488 cells/mm3. Neutropenia was not clearly associated with antibiotic prophylactic therapy or immunoglobulin levels, while infections were associated with neutropenia in the majority of episodes. Most acute neutropenia episodes were mild or moderate, except in CVID patients who present more severe neutropenia. Although IVIG may have contributed to reducing the severity of neutropenia, it does not prevent its occurrence in all patients. In conclusion, primary immunodeficient patients, even submitted to IVIG replacement therapy, must be regularly evaluated for neutropenia in order to minimize the risk of infections and its appropriate approach.
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Clinical spectrum of X-linked hyper-IgM syndrome
Jacov Levy, Teresa Espanol-Boren, Carolin Thomas et al. · The Journal of Pediatrics · 1997 · 660 citations
The X-Linked Hyper-IgM Syndrome
Jerry A. Winkelstein, Mary C. Marino, Hans D. Ochs et al. · Medicine · 2003 · 504 citations
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Nathan And Oski's Hematology Of Infancy And Childhood
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