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Hematology Reports
Volume 7
Issue 2
10.4081/hr.2015.5841
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Article

Prognostic Factors and Outcomes of Adult-Onset Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 34 Cases

by
Masafumi Oto
1,*,
Kanako Yoshitsugu
1,
Shima Uneda
1,
Michiko Nagamine
2 and
Minoru Yoshida
1
1
Department of Hematology and Medical Oncology, Japanese Red Cross Kumamoto Hospital, 2-1-1, Nagamine-minami, Higashi-Ku, Kumamoto-City 861-8520, Japan
2
Department of Pathology, Japanese Red Cross Kumamoto Hospital, Kumamoto-City 861-8039, Japan
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2015, 7(2), 5841; https://doi.org/10.4081/hr.2015.5841
Submission received: 2 February 2015 / Revised: 18 May 2015 / Accepted: 29 May 2015 / Published: 18 June 2015

Abstract

Adult-onset hemophagocytic lymphohistiocytosis (HLH) has features that are distinct from that of HLH in pediatric patients. The clinical records at the Japanese Red Cross Kumamoto Hospital were reviewed. We retrospectively analyzed 34 patients who fulfilled the diagnostic criteria of HLH-2004. The median age of patients was 60.0 (range 15–86). Underlying diseases were diagnosed in 17 patients. They consisted of malignant lymphoma (n = 3), other neoplastic disease (n = 3), viral infection (n = 4), collagen vascular disease (n = 3), Kikuchi’s disease (n = 3) and drug (n = 1). Underlying diseases were not diagnosed in 17 patients despite examination. The treatments were steroids (n = 18), dexamethasone + cyclosporine A (CSA) + etoposide (n = 4), multidrug chemotherapy (n = 2), steroids and CSA (n = 3). Eleven patients died during observation. In a multivariate analysis, the significant predictor for death was age at onset (hazard ratio, 1.22; 95%CI, 1.02–1.44; P = 0.027). Autopsy was performed in 4 cases, but the underlying disease remained unknown in 3 of those cases. Adult-onset HLH has high diversity and various outcomes. The mechanism of adult-onset HLH is not fully understood and further research is required.
Keywords: hemophagocytic lymphohistiocytosis; adult; prognosis; autopsy hemophagocytic lymphohistiocytosis; adult; prognosis; autopsy

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MDPI and ACS Style

Oto, M.; Yoshitsugu, K.; Uneda, S.; Nagamine, M.; Yoshida, M. Prognostic Factors and Outcomes of Adult-Onset Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 34 Cases. Hematol. Rep. 2015, 7, 5841. https://doi.org/10.4081/hr.2015.5841

AMA Style

Oto M, Yoshitsugu K, Uneda S, Nagamine M, Yoshida M. Prognostic Factors and Outcomes of Adult-Onset Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 34 Cases. Hematology Reports. 2015; 7(2):5841. https://doi.org/10.4081/hr.2015.5841

Chicago/Turabian Style

Oto, Masafumi, Kanako Yoshitsugu, Shima Uneda, Michiko Nagamine, and Minoru Yoshida. 2015. "Prognostic Factors and Outcomes of Adult-Onset Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 34 Cases" Hematology Reports 7, no. 2: 5841. https://doi.org/10.4081/hr.2015.5841

APA Style

Oto, M., Yoshitsugu, K., Uneda, S., Nagamine, M., & Yoshida, M. (2015). Prognostic Factors and Outcomes of Adult-Onset Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 34 Cases. Hematology Reports, 7(2), 5841. https://doi.org/10.4081/hr.2015.5841

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