Ateraciones en el extendido de sangre periferica en las inmunodeficiencias primarias / Alterations in the peripheal blood spreading in primary inmunodeficiencies

Los datos obtenidos a partir del extendido de sangre periférica son esenciales para el diagnóstico de diversas enfermedades, como en el caso particular de las inmunodeficiencias primarias, donde son considerados un parámetro clave. Las inmunodeficiencias primarias son un grupo de trastornos heterogéneos y complejos del sistema inmunológico, frecuentemente asociados con anormalidades en el número y/o en la morfología de las células sanguíneas. Estas alteraciones pueden ser observadas en un extendido de sangre periférica y fácilmente asociadas con algunas inmunodeficiencias en particular, brindando orientación para el diagnóstico y el tratamiento oportuno y adecuado de los pacientes. Esta revisión resume las alteraciones que pueden encontrarse en el estudio del extendido de sangre periférica de los pacientes con inmunodeficiencias primarias, recalcando la importancia de esta prueba en la práctica clínica, especialmente en la evaluación de los pacientes con alteraciones en el sistema inmune.

Metabolic and hematologic changes occurring after rapid intravenous infusion of gammaglobulin in patients with antibody deficiency syndromes

We wished to investigate whether increased IgG infusion rates are associated with metabolic and hematologic changes in pediatric patients with antibody deficiency syndromes. Methods: We studied 7 patients (2-16 years old) with primary antibody deficiencies who had been on regular IgG replacement treatment, 350-600 mg/kg/dose every 3 weeks with a 3 per cent IVIG preparation, for periods ranging from 6 months to 4 years. Initially, the IgG concentration of IVIG preparations was increased to 6, 9 and 12 per cent in consecutive infusions at a constant IgG infusion rate of 4 mg/kg/min. Subsequently the infusion rates were increased to 8, 12, and 16 mg/kg/min using the IVIG 12 per cent preparation. Results: Clinically, all patients tolerated increases in IVIG concentrations white the infusion rate was 4 mg/kg/min. However, 3 patients presented side effects when the infusion rate was increased to 8 and 16 mg/kg/min. Conclusion: We conclude that metabolic and hematologic sides effects, occur with rapid infusion of IVIG even in patients who tolerate the increased infusion rate clinically. The advantages of using high infusion rates have to be re-evaluated.

Hipogamaglobulinemia com imunoglobulina M normal ou aumentada: relato de casos / Hypergammaglobulinemia with normal or elevated immunoglobulinemia - a case report

A hipogamaglobulinemia com imunoglobulina M (IgM) normal ou elevada é uma imunodeficiência raramente observada, sendo também conhecida com o nome de síndrome de Hiper IgM. Esta síndrome é diagnosticada em pacientes portadores de níveis séricos de imunoglobulinas (IgG e IgA) diminuídos, com níveis de imunoglobulina M (IgM) normais ou elevados. O objetivo do presente relato é descrever as características clínico-laboratoriais e a evoluçäo de dois pacientes portadores desta síndrome, com idades de 2 anos e 10 meses e 10 anos, acompanhados na Unidade de Alergia e Imunologia do ICr HCFMUSP. As manifestaçöes clínicas foram precoces, com infecçöes recorrentes de vias aéreas e trato gastrointestinal. A avaliaçäo imunológica encontramos diminuiçäo dos níveis de IgG e IgA, com níveis de IgM normais no primeiro paciente e elevados (acima do percentil 97,5) no segundo paciente. Após o diagnóstico, os pacientes receberam gamaglobulina, antibióticos e orientaçäo quanto à nutriçäo e ambiente físico, com melhora evidente dos sintomas apresentados e sem o desenvolvimento de complicaçöes. Os autores ressaltam a importância do diagnóstico precoce da síndrome de Hiper IgM para tratamento adequado e controle dos processos infecciosos.

Effect of zinc supplementation on zinc status, copper status and cellular immunity in elderly patients with diabetes mellitus.

This preliminary study was to investigate the effects of zinc supplementation on Zn status, Cu status, serum macrominerals and lymphocyte subsets in elderly diabetic patients. The results of Zn supplementation can correct plasma Zn levels to normal values. However, this treatment did not affect the cellular Zn, copper status and serum macrominerals. Enhancement of the percentage of CD4 cells was observed after Zn therapy but had no effect on the percentage of CD8 cells and CD4/CD8 ratios. Our finding implicated that zinc supplementation might be useful to enhance the immune status in these patients.

Immunoglobulin deficiency.

Twenty three patients with primary immunoglobulin(Ig) deficiency were seen during the last ten years. Nine had hypogamma globulinemia (hypo-Ig) and the rest, selective Ig deficiency. Most were in pediatric age group. There was preponderance of males with only one female. Clinical symptoms pertaining to gastrointestinal and sinupulmonary infections were most common. Complete absence of B cells was not observed in any patient with hypogammaglobulinemia. They could be typed as physiological in one patient, X-linked immunodeficiency in 2 patients and common variable immunodeficiency in the remaining six. Three patients with selective IgA deficiency were above 20 years of age. Two had only secretory IgA deficiency, confirmed by jejunal fluid examination and the rest had both secretory and serum IgA deficiency. Low IgM was seen in one patient. We see a spectrum of immunoglobulin deficiencies varying from subtle defects like absence of secretory IgA only, to severe depletion of all immunoglobulins. Therapy is still not ideal due to economic reasons.