Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: response to HLH-04 treatment protocol

Boletín Médico del Hospital Infantil de México

卷:73

José Arellano-Galindo¹  Elva Jiménez-Hernández²  Paloma del Rocío Loza-Santiaguillo²  Beatriz Hernández-Sánchez²  María Angélica Martínez-Martell²  Octavio Martínez-Villegas²  Berenice Sánchez-Jara²  Eduardo Pedro-Matías² 

[1] Laboratorio de Microbiología y Enfermedades Infecciosas, Departamento de Infectología, Hospital Infantil de México Federico Gómez, ;

[2] Servicio de Hematología Pediátrica, Unidad Médica de Alta Especialidad, Hospital General, Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, ;

关键词: Hemophagocytic lymphohistiocytosis;    Hemophagocytic syndrome;    Epstein-Barr virus;    Protocol HLH-04;   

DOI  :  10.1016/j.bmhimx.2015.12.007

来源: DOAJ

【 摘 要 】

Introduction: Hemophagocytic syndrome, macrophage activation syndrome, reactive histiocytosis or hemophagocytic lymphohistiocytosis (HLH) represent a group of diseases whose common thread is reactive or neoplastic mononuclear phagocytic system cells and dendritic cell proliferation.Clinical case: We present a case of an HLH probably associated with Epstein-Barr virus (EBV) in a 4-year-old male patient treated with HLH-04 protocol. Viral etiology in HLH is well accepted. In this case, clinical picture of HLH was assumed secondary to EBV infection because IgM serology at the time of clinical presentation was the only positive factor in the viral panel.Conclusions: Diagnosis of HLH is the critical first step to successful treatment. The earlier it is identified, the less the tissue damage and reduced risk of multiple organ failure, which favors treatment response.

【 授权许可】

Unknown