RESUMEN
We describe the case of an infant with recurrent episodes of staphylococcal skin abscess and subsequent lethal pneumococcal meningitis/septicemia due to interleukin-1 receptor-associated kinase 4 (IRAK-4) deficiency. In this case, systemic signs of inflammatory response were poor and delayed. Among all other reported cases of IRAK-4 deficiency, none involved severe viral or fungal disease, and the range of infecting bacteria was narrow.
Asunto(s)
Absceso/complicaciones , Síndromes de Inmunodeficiencia/complicaciones , Meningitis Neumocócica/complicaciones , Sepsis/etiología , Infecciones Estafilocócicas/complicaciones , Staphylococcus aureus/aislamiento & purificación , Absceso/diagnóstico , Absceso/microbiología , Resultado Fatal , Femenino , Estudios de Seguimiento , Humanos , Síndromes de Inmunodeficiencia/diagnóstico , Lactante , Quinasas Asociadas a Receptores de Interleucina-1 , Meningitis Neumocócica/diagnóstico , Meningitis Neumocócica/microbiología , Enfermedades de Inmunodeficiencia Primaria , Sepsis/diagnóstico , Sepsis/microbiología , Infecciones Estafilocócicas/diagnóstico , Infecciones Estafilocócicas/microbiologíaAsunto(s)
Síndrome de Fuga Capilar/diagnóstico , Citocinas/sangre , Linfoma Anaplásico de Células Grandes/complicaciones , Proteínas Tirosina Quinasas Receptoras/metabolismo , Adolescente , Quinasa de Linfoma Anaplásico , Síndrome de Fuga Capilar/etiología , Factor Estimulante de Colonias de Granulocitos/sangre , Humanos , Interferón gamma/sangre , Interleucina-10/sangre , Interleucina-6/sangre , Linfoma Anaplásico de Células Grandes/sangre , Linfoma Anaplásico de Células Grandes/enzimología , MasculinoRESUMEN
In this article, we first report a case of recurrent paroxysmal cold hemoglobinuria with serologic confirmation. On 2 occasions, the Donath-Landsteiner (DL) antibodies belonged to an IgM subclass and showed neither anti-P nor anti-I specificity. Furthermore, it is very interesting that the temperature thresholds of DL antibodies were different on each occasion. Although acute paroxysmal cold hemoglobinuria is considered to be self-limited and transient, we should be careful of its possible recurrence. DL tests must be repeated after the complete recovery from the first episode, with careful attention to several possible causes of false-negative DL tests.
