II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies

Abstract

In the last few years, new primary immunodeficiencies and genetic defects have been described. Recently, immunoglobulin products with improved compositions and for subcutaneous use have become available in Brazil. In order to guide physicians on the use of human immunoglobulin to treat primary immunodeficiencies, based on a narrative literature review and their professional experience, the members of the Primary Immunodeficiency Group of the Brazilian Society of Allergy and Immunology prepared an updated document of the 1st Brazilian Consensus, published in 2010. The document presents new knowledge about the indications and efficacy of immunoglobulin therapy in primary immunodeficiencies, relevant production-related aspects, mode of use (routes of administration, pharmacokinetics, doses and intervals), adverse events (major, prevention, treatment and reporting), patient monitoring, presentations available and how to have access to this therapeutic resource in Brazil.

Neuroendocrine Tumor in a Child With Common Variable Immunodeficiency

“…5 The replacement with human immunoglobulin allowed for the defense against such agents, and the patient no longer had pneumonia, as described in the literature. 4 The patient also had a laboratory picture of IgA deficiency, which is part of CVID. The lack of IgA can lead to tonsillitis, otitis, recurrent diarrhea and giardiasis, which were not presented by the patient in question.…”

“…3 CVID is defined as decreased immunoglobulin G (IgG) and IgA, or IgM and specific antibody deficiency after ruling out other causes of hypogammaglobulinemia. 4 For the class change from IgM to IgG and IgA, adhesion molecules expressed on T helper and B lymphocytes are needed, respectively: CD40L to CD40; Inducible Costimulatory molecule (ICOS) to ICOS-L; B-cell Activating Factor (BAFF) receptor to Transmembrane Activator and Calcium-modulator Ligand (TACI). 5 For the final differentiation of B lymphocyte into plasmocyte, the presence of B differentiation groups is essential: CD19, CD20, CD21, CD81.…”

Manifestaciones clínicas y diagnóstico tardío de la inmunodeficiencia común variable

“…La dosis habitual recomendada es de 400 a 800 mg/kg, con intervalos de tres a cuatro semanas, con la que se obtiene control clínico y titulaciones séricas estables de IgG. 4 Las dosis administradas al paciente estaban de acuerdo con esas recomendaciones y fueron eficaces para prevenir nuevas neumonías. El tratamiento de la giardiasis y el refuerzo de la higiene personal y ambiental hicieron desaparecer la diarrea.…”

“…El tratamiento del paciente con IDCV y con neumonías de repetición se realiza con reposición de inmunoglobulina humana, lo que mejora la calidad de vida del paciente y hasta la supervivencia. 4 Para el manejo de la deficiencia de la IgA es necesario hacer un refuerzo de la higiene personal y ambiental. 5 Se describe un paciente con manifestaciones clínicas y diagnóstico tardíos de IDCV.…”

Comment to: II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies. einstein (São Paulo). 2017;15(1):1-16

“…We would like to update the readership of einstein (São Paulo) on the information given in page 6, regarding hyaluronidase facilitated subcutaneous immunoglobulin, in the article entitled II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies published in this journal in volume 15 issue 1, 2017. ( 1 )…”