A long-lasting febrile syndrome

Authors

  • Elizabeth Lorenzo-Hernández Servicio de Medicina Interna, Hospital Regional Universitario de Málaga, Málaga, Spain
  • Fernando Salgado-Ordoñez Servicio de Medicina Interna, Hospital Regional Universitario de Málaga, Málaga, Spain
  • Iván Pérez-de-Pedro Servicio de Medicina Interna, Hospital Regional Universitario de Málaga, Málaga, Spain
  • Gloria Millán-García Unidad de Cuidados Médicos y Paliativos, Centro Asistencial San Juan de Dios, Málaga, Spain
  • Ricardo Gómez-Huelgas Servicio de Medicina Interna, Hospital Regional Universitario de Málaga, Málaga, Spain

DOI:

https://doi.org/10.32818/reccmi.a5n1a10

Keywords:

adult-onset Still’s disease, fever, ferritin, hemophagocytosis, rash, splenomegaly, hemophagocytic syndrome.

Abstract

It is necessary to include the hemophagocytic syndrome within the differential diagnosis of intermediate-long febrile syndrome. This is a rare and life-threatening entity due to immune hyperactivation. It is characterized by sepsis-like manifestations and it shows hemaphagocytosis (ingestion of blood cells and their precursors by macrophages). It can be primary or secondary to some disease, such as an infection or a neoplasia, as more frequent entities, or to an autoimmune disease, such as the adult-onset Still’s disease, as it was in our case. The secondary entities must be searched for to start the targeted treatment. It is essential an early diagnosis of hemophagocytic syndrome due to its severity and the mortality resulting from a delay in treatment and to the low incidence.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

References

Emenegger U, Schaer DJ, Larroche C, Neftel KA. Haemophagocytic syndromes in adults: current concepts and challenges ahead. Swiss Med Wkly. 2005; 135: 299-314. Disponible en: https://smw.ch/en/resource/jf/journal/file/view/article/smw/en/smw.2005.10976/d9988fc7dda9215cd876ebe-73c05ad9c70ac9e95/smw.2005.10976.pdf (último acceso: 11 de febrero de 2020).

Yildiz H, Van Den Neste E, Defour JP, Danse E, Yombi JC. Adult haemophagocytic lymphohistiocytosis: a review. QJM. 2020 Jan 14. pii: hcaa011. https://doi.org/10.1093/qjmed/hcaa011.

Ruscitti P, Iacono D, Ciccia F, Emmi G, Cipriani P, Grembiale RD, et al. Macrophage activation syndrome in patients affected by adult-onset Still disease: analysis of survival rates and predictive factors in the Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale Cohort. J Rheumatol. 2018 Jun; 45(6): 864-872. https://doi.org/10.3899/jrheum.170955.

Bae CB, Jung JY, Kim HA, Suh CH. Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features, predictive factors, and prognosis in 21 patients. Medicine (Baltimore). 2015; 94(4): e451. https://doi.org/10.1097/MD.0000000000000451.

Zhang XH, Han YM, Wang WW, Cheng H, Zhu KJ. Hemophagocytic syndrome secondary to adult-onset Still's disease but very similar to lymphoma. Int J Clin Exp Pathol. 2012; 5(4): 377-381. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3365815 (último acceso: marzo 2020).

Mimura T, Kondo Y, Ohta A, Iwamoto M, Ota A, Okamoto N, et al. Evidence-based clinical practice guideline for adult Still's disease. Mod Rheumatol. 2018; 28(5): 736-757. https://doi.org/10.1080/14397595.2018.1465633.

Yoo DH. Treatment of adult-onset still's disease: up to date. Expert Rev Clin Immunol. 2017 Sep; 13(9): 849-866. https://doi.org/10.1080/1744666X.2017.1332994.

Mehta MV, Manson DK, Horn EM, Haythe J. An atypical presentation of adult-onset Still's disease complicated by pulmonary hypertension and macrophage activation syndrome treated with immunosuppression: a case-based review of the literature. Pulm Circ. 2016; 6(1): 136-142. https://doi.org/10.1086/685112.

Published

2020-04-30

How to Cite

1.
Lorenzo-Hernández E, Salgado-Ordoñez F, Pérez-de-Pedro I, Millán-García G, Gómez-Huelgas R. A long-lasting febrile syndrome. Rev Esp Casos Clin Med Intern [Internet]. 2020 Apr. 30 [cited 2024 Jul. 22];5(1):27-30. Available from: https://www.reccmi.com/RECCMI/article/view/469

Most read articles by the same author(s)

<< < 1 2