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Vol. 214. Issue 6.
Pages 320-327 (August - September 2014)
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Vol. 214. Issue 6.
Pages 320-327 (August - September 2014)
Clinical up-date
Hemophagocytic lymphohistiocytosis associated with viral infections: Diagnostic challenge and therapeutic dilemma
Linfohistiocitosis hemofagocítica asociada a infecciones virales: reto diagnóstico y dilema terapéutico
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J.L. Mostaza-Fernándeza,
Corresponding author
jlmostaza@yahoo.es

Corresponding author.
, J. Guerra Lasoa, D. Carriedo Uleb, J.M.G. Ruiz de Moralesc
a Servicio de Medicina Interna, Complejo Asistencial Universitario de León, León, Spain
b Servicio de UCI, Complejo Asistencial Universitario de León, León, Spain
c Sección de Inmunología Clínica. Complejo Asistencial Universitario de León, León, Spain
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Tables (4)
Table 1. Classification of hemophagocytic lymphohistiocytosis (HLH).
Table 2. Secondary hemophagocytic lymphohistiocytosis. Triggers.
Table 3. Diagnostic criteria.
Table 4. Microbiological tests to consider in patients with HLH.
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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 toward treating the underlying disease and to suppressing the exaggerated inflammatory response through the use of immunosuppressive agents.

Keywords:
Hemophagocytic lymphohistiocytosis
Perforin
Hemophagocytosis
Viral infections
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.

Palabras clave:
Linfohistiocitosis hemofagocítica
Perforina
Hemofagocitosis, Infecciones virales

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