A Clinical Analysis of 52 Adult Patients With Hemophagocytic Syndrome: The Prognostic Significance of the Underlying Diseases View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2001-08-16

AUTHORS

Naoto Takahashi, Akihiko Chubachi, Masaaki Kume, Yoshiaki Hatano, Atsushi Komatsuda, Yoshinari Kawabata, Norimitsu Yanagiya, Yoshikazu Ichikawa, Akira B. Miura, Ikuo Miura

ABSTRACT

We retrospectively analyzed 52 adult patients with hemophagocytic syndrome (HPS). The underlying diseases were heterogeneous, including malignant lymphoma (lymphoma-associated hemophagocytic syndrome [LAHS]) in 26 patients, systemic lupus erythematosus in 3 patients, viral infections in 7 patients, and bacterial or fungal infections in 6 patients. More than 83% of patients received prednisolone as an initial treatment. Multiple-agent chemotherapies (cyclophosphamide, doxoru-bicin, and vincristine) were administered to 96% of LAHS patients after a histopathological diagnosis of lymphoma. HPSs were controllable and remissions were achieved except for those patients with LAHS, fulminant Epstein-Barr virus-ssociated HPS, and an immunosuppressive state. Twenty-one (81%) of the LAHS patients had uncontrollable HPS and died of multiple organ failure and disseminated intravascular coagulation.The median survival time of LAHS patients was 83 days. In contrast, 3 (12%) of the other HPS patients died of multiple organ failure within 44 days.The clinical manifestations and the laboratory findings of LAHS and the other HPSs were too variable to establish the prognosis based only on the findings at the onset of HPS. The prognostic factors of adult HPS were found to be the underlying diseases, notably malignant lymphoma and infections, accompanied by the immunosuppressive state. More... »

PAGES

209-213

References to SciGraph publications

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/bf02982007

DOI

http://dx.doi.org/10.1007/bf02982007

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1023328621

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/11594524


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