[Acquired hemophagocytic syndrome treated with HLH 94-04 chemotherapy protocol: Report of four cases].

Abstract

Hemophagocytic syndrome is a severe condition of excessive immune activation that has a high mortality in the absence of treatment. The syndrome is classified as primary if associated with congenital or hereditary problems, or secondary/acquired if associated with infectious, autoimmune or oncology diseases. We report four adult cases of the syndrome, one with viral, two with autoimmune and one with idiopathic causes who were successfully treated with HLH 94-04 chemotherapy protocol. Our experience shows that a high index of suspicion, early diagnosis and an opportune therapy are essential in the treatment of this disease.

• Authors: Nicole Beffermann C, Javier Pilcante S, Mauricio Ocqueteau T, Mauricio Sarmiento M
• Journal: Revista medica de Chile (Rev Med Chil) Vol. 143 Issue 9 Pg. 1172-8 (Sep 2015) ISSN: 0717-6163 [Electronic] Chile
• Vernacular Title: Síndrome hemofagocítico adquirido: reporte de casos de cuatro pacientes adultos tratados con protocolo HLH 94-04 y revisión de la literatura.
• PMID: 26530200 (Publication Type: Case Reports, English Abstract, Journal Article, Review)

Chemical References

• Immunosuppressive Agents
• Etoposide
• Dexamethasone
• Cyclosporine
• Ferritins
• Methotrexate

Topics

• Adult
• Cyclosporine (therapeutic use)
• Dexamethasone (therapeutic use)
• Drug Therapy, Combination (methods)
• Early Diagnosis
• Etoposide (therapeutic use)
• Female
• Ferritins (blood)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Lymphohistiocytosis, Hemophagocytic (diagnosis, drug therapy, physiopathology)
• Male
• Methotrexate (therapeutic use)
• Young Adult