Síndrome hemofagocítico asociado a virus de Epstein-Barr. Descripción de un caso y tratamiento en la Unidad de Reanimación / Hemophagocytic syndrome associated with Epstein-Barr virus. Presentation of a case and treatment in the Resuscitation Unit

Exponemos el caso clínico de un paciente varón de 17 años que ingresa en la Unidad de Reanimación con el diagnóstico de síndrome hemofagocítico asociado a infección por el virus de Epstein-Barr con evolución desfavorable. El síndrome hemofagocítico es un síndrome de activación inmune patológica debido a una producción/modulación incontrolada de algunas citocinas. Su cuadro clínico se define según los criterios de consenso HLH-2004, no son patognomónicos, y muchas veces aparecen secuencialmente, por lo que su sospecha debe seguirse de tratamiento de soporte agresivo combinado precozmente con el tratamiento específico del factor desencadenante, ya que es la única forma de mejorar la supervivencia en pacientes con fallo orgánico múltiple por esta causa (AU)

We report the case of a 17 year old male patient, who was admitted to the Resuscitation Unit with the diagnosis of hemophagocytic syndrome, associated with infection by Epstein-Barr virus with unfavorable outcome. Hemophagocytic syndrome is a pathological immune activation syndrome due to the production/uncontrolled modulation of some cytokines. Its clinical signs and symptoms, defined by consensus criteria HLH-2004, are not pathognomonic, and often appear sequentially, thus suspicion should be followed by aggressive supportive therapy combined with early specific treatment of the triggering factor, as it is the only way to improve survival in patients with multiple organic failure as a result (AU)

Tumor cerebral no conocido que se presenta con compresión del tronco cerebral tras punción dural accidental / Unknown intracerebral tumour presenting as brainstem compression following unintentional dural puncture

Se trata de una primigrávida de 36 años de edad en su semana 41 de gestación con antecedentes de hiperemesis gravídica. Durante la analgesia epidural se produjo una punción dural accidental. En el posparto presentó cefalea persistente, tratada mediante analgésicos orales, cafeína, fluidoterapia y tetracosáctido, rechazando el parche hemático epidural. En el séptimo día posparto la paciente reingresó en el Servicio de Urgencias con deterioro del nivel de conciencia y datos de compresión del tronco cerebral. La tomografía computarizada y la resonancia magnética craneales mostraron un tumor de fosa posterior. Se realizó una craneotomía con carácter de urgencia, con recuperación neurológica completa. Resaltamos la importancia del diagnóstico diferencial de la cefalea pospunción dural y destacamos los signos de alarma ante los pacientes que no responden a los tratamientos convencionales (AU)

A 36-year old primigravid of 41 weeks gestation was admitted to the labour ward. Her past medical history included hyperemesis gravidarum and migraine. An accidental dural puncture occurred during labour epidural analgesia. In the postpartum period she presented with continuous headache, and was treated with oral analgesics, oral caffeine, fluid therapy, and tetracosactide. She refused an epidural blood patch. On the seventh day postpartum, the patient was re-admitted to the Emergency Department with decreased level of consciousness and signs of brainstem compression. Cranial computed tomography and magnetic resonance imaging showed a posterior fossa tumour. An emergency craniotomy was performed with complete neurological recovery. This case emphasises the need to consider the differential diagnoses of post-dural puncture headache and to highlight the warning signs in patients who do not respond despite treatment with conventional therapy (AU)

[Hemophagocytic syndrome associated with Epstein-Barr virus. Presentation of a case and treatment in the Resuscitation Unit]. / Síndrome hemofagocítico asociado a virus de Epstein-Barr. Descripción de un caso y tratamiento en la Unidad de Reanimación.

We report the case of a 17 year old male patient, who was admitted to the Resuscitation Unit with the diagnosis of hemophagocytic syndrome, associated with infection by Epstein-Barr virus with unfavorable outcome. Hemophagocytic syndrome is a pathological immune activation syndrome due to the production/uncontrolled modulation of some cytokines. Its clinical signs and symptoms, defined by consensus criteria HLH-2004, are not pathognomonic, and often appear sequentially, thus suspicion should be followed by aggressive supportive therapy combined with early specific treatment of the triggering factor, as it is the only way to improve survival in patients with multiple organic failure as a result.

[Unknown intracerebral tumour presenting as brainstem compression following unintentional dural puncture]. / Tumor cerebral no conocido que se presenta con compresión del tronco cerebral tras punción dural accidental.

A 36-year old primigravid of 41 weeks gestation was admitted to the labour ward. Her past medical history included hyperemesis gravidarum and migraine. An accidental dural puncture occurred during labour epidural analgesia. In the postpartum period she presented with continuous headache, and was treated with oral analgesics, oral caffeine, fluid therapy, and tetracosactide. She refused an epidural blood patch. On the seventh day postpartum, the patient was re-admitted to the Emergency Department with decreased level of consciousness and signs of brainstem compression. Cranial computed tomography and magnetic resonance imaging showed a posterior fossa tumour. An emergency craniotomy was performed with complete neurological recovery. This case emphasises the need to consider the differential diagnoses of post-dural puncture headache and to highlight the warning signs in patients who do not respond despite treatment with conventional therapy.