Linfohistiocitosis hemofagocítica asociada a infecciones virales: reto diagnóstico y dilema terapéutico / Hemophagocytic lymphohistiocytosis associated with viral infections: diagnostic challenges and therapeutic dilemmas
Rev. clín. esp. (Ed. impr.)
; 214(6): 320-327, ago.-sept. 2014.
Article
in Spanish
| IBECS
| ID: ibc-125518
Responsible library:
ES1.1
Localization: BNCS
RESUMEN
La linfohistiocitosis hemofagocítica es un síndrome clinicopatológico de evolución potencialmente fatal, en el que una respuesta inmune no controlada e ineficaz conduce a hiperinflamación. Puede aparecer como una enfermedad familiar o esporádica, asociado a diferentes factores desencadenantes infecciones, neoplasias, enfermedades autoinmunes o inmunodeficiencias adquiridas, pero la asociación más consistente es con infecciones virales, especialmente el virus de Epstein-Barr. Las principales características clínicas son fiebre, disfunción hepática, coagulopatía y pancitopenia. El diagnóstico es difícil debido a la rareza de este síndrome y a la falta de especificidad de los hallazgos clínicos, sin embargo, un diagnóstico y tratamiento precoces son importantes para disminuir la mortalidad. El tratamiento debe ser dirigido al control de la enfermedad subyacente y a suprimir la respuesta inflamatoria exagerada mediante el uso de inmunosupresores (AU)
ABSTRACT
Hemophagocytic lymphohistiocytosis is a frequently fatal clinicopathologic syndrome in which an uncontrolled and ineffective immune response leads to severe hyperinflammation. It may occur as either a familial disorder or a sporadic condition in association with a variety of triggers infections, malignancies, autoimmune diseases, and acquired immune deficiencies. However, the most consistent association is with viral infections, especially Epstein-Barr virus. The main clinical features are fever, liver dysfunction, coagulation abnormalities and pancytopenia. Early diagnosis and treatment are important to reducing mortality, but the diagnosis is difficult because of the rarity of the syndrome and the lack of specificity of the clinical findings. Treatment should be directed towards treating the underlying disease and to suppressing the exaggerated inflammatory response through the use of immunosuppressive agents (AU)
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Collection:
National databases
/
Spain
Health context:
SDG3 - Target 3.4 Reduce premature mortality due to noncommunicable diseases
Health problem:
Digestive System Diseases
/
Other Blood Disorders
Database:
IBECS
Main subject:
Virus Diseases
/
Epstein-Barr Virus Infections
/
Lymphohistiocytosis, Hemophagocytic
/
Perforin
/
Immunosuppressive Agents
Type of study:
Diagnostic study
/
Etiology study
/
Risk factors
/
Screening study
Limits:
Female
/
Humans
/
Male
Language:
Spanish
Journal:
Rev. clín. esp. (Ed. impr.)
Year:
2014
Document type:
Article
Institution/Affiliation country:
Complejo Asistencial Universitario de León/España