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Inicio Medicina Clínica (English Edition) Hemophagocytic lymphohistiocytosis: Analysis of 18 cases
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Vol. 147. Issue 11.
Pages 495-498 (December 2016)
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Vol. 147. Issue 11.
Pages 495-498 (December 2016)
Clinical report
DOI: 10.1016/j.medcle.2016.12.025
Hemophagocytic lymphohistiocytosis: Analysis of 18 cases
Linfohistiocitosis hemofagocítica: análisis de 18 casos
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Pilar Hernández-Jiméneza,
Corresponding author
pilihj@hotmail.com

Corresponding author.
, Carmen Díaz-Pedrochea, Jaime Laureiroa, Olaya Madrida, Estela Martínb, Carlos Lumbrerasa
a Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, Spain
b Servicio de Hematología, Hospital Universitario 12 de Octubre, Madrid, Spain
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Tables (3)
Table 1. 2004 HLH-trial diagnostic criteria.
Table 2. Baseline characteristics of patients and clinical, laboratory and bone marrow findings.
Table 3. Aetiology, treatment and prognosis of hemophagocytic lymphohistiocytosis in 18 adult patients.
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Abstract
Background and objective

Hemophagocytic lymphohistiocytosis (HLH) is a serious condition, caused by an improper regulation of the immune response to different stimuli of the immune system. Early diagnosis and treatment are a challenge for the clinician.

Patients and method

We conducted a retrospective study at our institution between 2010 and 2015, of adult patients diagnosed with HLH, in accordance with the criteria of the Histiocyte Society, analysing their clinical characteristics, diagnostic and etiological studies and the outcome.

Results

Eighteen patients were analysed. Median time to diagnosis was 24 days. We found neoplastic aetiology in 8 cases (7 hematologic), while it was infection-related in 6 (4 visceral leishmaniasis), and an inflammatory disease in one. In the remaining 3, an underlying cause for the HLH was not found. Course of treatment was corticosteroids in 16 patients, associated with cyclosporine in 2 of them, one received immunoglobulins, while another received etoposide with tacrolimus.

Conclusions

We emphasize the scarce use of etoposide therapy, the currently recommended treatment. Overall mortality was 44%, mainly associated with neoplastic aetiology (67 compared to 16.6% mortality in infection-related aetiology, P<.05).

Keywords:
Hemophagocytic syndrome
Macrophage activation syndrome
Adult
Clinical presentation
Aetiology
Outcome
Resumen
Antecedentes y objetivo

La hemophagocytic lymphohistiocytosis (HLH, «linfohistiocitosis hemofagocítica») es una entidad grave, producida por una incorrecta regulación de la respuesta inmunológica frente a diversos estímulos del sistema inmunitario. Su diagnóstico y tratamiento precoz suponen un reto para el clínico.

Pacientes y método

Hemos realizado un estudio descriptivo retrospectivo de los pacientes adultos diagnosticados de HLH, según los criterios de la Histiocyte Society, entre los años 2010 y 2015 en nuestra institución, analizando sus características clínicas, el estudio diagnóstico-etiológico y su evolución.

Resultados

Se analizaron 18 pacientes. La mediana de tiempo al diagnóstico fue de 24 días. La etiología fue neoplásica en 8 casos (hematológica en 7), infecciosa en 6 (leishmaniasis visceral en 4), inflamatoria en uno, y en los 3 restantes, idiopática. Se realizó tratamiento en 16 pacientes con corticoides, asociando ciclosporina en 2, inmunoglobulinas en uno, y etopósido con tacrolimus en otro.

Conclusiones

Destacamos la escasa utilización de etopósido en el tratamiento dirigido, el actualmente recomendado. La mortalidad global fue del 44%, asociada a la etiología neoplásica principalmente (67 frente a 16,6% de mortalidad en la etiología infecciosa, p<0,05).

Palabras clave:
Síndrome hemofagocítico
Síndrome de activación macrofágica
Adulto
Presentación clínica
Etiología
Pronóstico

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