Assuntos
Herpesvirus Humano 4/isolamento & purificação , Mononucleose Infecciosa/diagnóstico , Linfonodos/microbiologia , Biópsia , Feminino , Humanos , Recém-Nascido , Mononucleose Infecciosa/microbiologia , Mononucleose Infecciosa/transmissão , Masculino , Gravidez , Complicações Infecciosas na Gravidez/imunologiaRESUMO
Four Venezuelan patients with the autosomal recessive Chediak-Higashi syndrome (CHS) were studied. The results confirm the severe reduction in natural killer (NK) cell activity, as previously described and showed also a decline in the activity of cells involved in antibody-dependent cellular cytotoxicity (ADCC). No defect was found in the production of immunoglobulins and of specific antibodies to measles, varicella, herpes simplex, and cytomegalo viruses. Two of the patients had extremely high antibody titers to the Epstein-Barr virus (EBV) specific viral capsid antigen (VCA), to the restricted (R) component of the EBV-induced early antigen complex, and to the EBV-associated nuclear antigen (EBNA). These two patients had enlarged livers, spleens, and lymph nodes indicative of the lymphoproliferative phase. The other two patients were initially negative for all EBV-associated antibodies but seroconverted subsequently and, in the course of a year, also developed high antibody titers to VCA and R. In one of these patients the primary infection was accompanied by moderate signs of infectious mononucleosis (IM) followed after more than 6 months by persistent hepatosplenomegaly. The other patient also developed signs of a lymphoproliferative syndrome with hepatosplenomegaly and jaundice and died 8 months later. Such high anti-R titers are seen frequently in Burkitt's lymphoma, but rarely in other conditions. It is likely that the high antibody titers reflect an increased production of VCA and R due to defective NK and ADCC cell activities so that productively infected B lymphocytes are no longer eliminated before they have synthesized maximal amounts of antigens. The high anti-EBNA titers suggest normal T lymphocyte function. The possibility that the accelerated, lymphoma-like phase of the CHS involves EBV-transformed cells is discussed.
Assuntos
Anticorpos Antivirais/biossíntese , Síndrome de Chediak-Higashi/imunologia , Infecções por Herpesviridae/imunologia , Células Matadoras Naturais/imunologia , Adulto , Anticorpos Antivirais/análise , Citotoxicidade Celular Dependente de Anticorpos , Proteínas Sanguíneas/análise , Síndrome de Chediak-Higashi/complicações , Síndrome de Chediak-Higashi/epidemiologia , Pré-Escolar , Citotoxicidade Imunológica , Feminino , Infecções por Herpesviridae/complicações , Herpesvirus Humano 4/imunologia , Humanos , Imunidade Celular , Lactente , Masculino , VenezuelaRESUMO
Three siblings developed severe (two) or fatal (one) infectious mononucleosis. This family differed from previously described kindreds with a susceptibility to overwhelming Epstein-Barr virus infections in that: (1) both males and females were affected; (2) they had a history of the recurrent bacterial infections; (3) they produced the full spectrum of antibodies to EBV in the expected range of titers; and (4) survivors recovered completely. Two of these youths, but not their parents or an unaffected sibling with mild IM, had a deficiency of natural killer activity that did not respond to preincubation of their peripheral blood mononuclear cells with interferon. NK activity may have an important role in controlling infections with EBV.