Causes and Risk Factors of Mortality in Adult Patients with Hemophagocytic Syndrome

Hye-In Kim; Shin-Woo Kim; Hyun-Ha Chang; Jong-Myung Lee; Neung-Su Kim; Ki-Tae Kwon; Seong-Yeol Ryu; Ji-An Hur

doi:10.3947/ic.2012.44.2.51

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Kim, Kim, Chang, Lee, Kim, Kwon, Ryu, and Hur: Causes and Risk Factors of Mortality in Adult Patients with Hemophagocytic Syndrome

Original Article

Infection & Chemotherapy

Infection & Chemotherapy 2012; 44(2): 51-55.

Published online: 30 April 2012

DOI: https://doi.org/10.3947/ic.2012.44.2.51

Causes and Risk Factors of Mortality in Adult Patients with Hemophagocytic Syndrome

Hye-In Kim¹, Shin-Woo Kim¹, Hyun-Ha Chang¹, Jong-Myung Lee¹, Neung-Su Kim¹, Ki-Tae Kwon², Seong-Yeol Ryu³, Ji-An Hur⁴

¹Department of Internal Medicine, School of Medicine, Kyungpook National University, Daegu, Korea.

²Department of Internal Medicine, Daegu Fatima Hospital, Daegu, Korea.

³Department of Internal Medicine, Keimyung University Dongsan Medical Centers, Daegu, Korea.

⁴Department of Internal Medicine, Yeungnam University Hospital, Daegu, Korea.

Correspondence to Shin-Woo Kim, M.D. Department of Internal Medicine, Kyungpook National University Hospital, 50 Samduk-dong 2-ga, Jung-gu, Daegu 700-721, Korea. Tel: +82-53-420-6525, Fax: +82-53-426-2046, ksw2kms@knu.ac.kr

Received 23 December 2011 Revised 27 February 2012 Accepted 28 February 2012

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background

Hemophagocytic syndrome (HS) is a distinct clinical entity characterized by high fever and hemophagocytosis with histiocytosis in tissue biopsy. We seldom encounter patients who suffer from unexplained, persistent fevers. Although there have been many studies about childhood HS, studies about adult HS are relatively rare. The causes and prognoses of HS in adults were evaluated in this study. We focused on infection-related HS.

Materials and Methods

We enrolled 41 adult patients with HS retrospectively from four hospitals in Kyungbuk province and Daegu city. The patients were diagnosed by bone marrow or liver biopsy, either of which showed hemophagocytosis with histiocytosis and had clinical findings consistent with HS. We explored the etiologies, clinical symptoms, laboratory findings, treatments, and outcomes of each case.

Results

The most common cause of HS was infection, such as the Epstein-Barr virus (EBV) or Mycobacterium tuberculosis. Old age and malignancy-associated HS had a poor prognosis. The overall mortality rate was 17.1%. Most patients survived after conservative therapy and the control of underlying diseases, in contrast to previous studies that showed a poor prognosis of infection-associated HS.

Conclusions

A proper investigation is crucial to determine the cause of HS in patients who have unexplained persistent fever and hemophagocytosis with histiocytosis in their tissue. Cases of infection-related HS are common, but physicians should consider undiagnosed malignancy that may be related to a poor prognosis. Treatments appropriate to the causes are important for better outcomes in adult HS.

Keywords: Hemophagocytic lymphohistiocytosis, Infection, Prognosis

Figures and Tables

Figure 1

Survival curve of 41 patients with hemophagocytic syndrome (log-rank test between malignancy group vs. non-malignancy: P<0.001).

Table 1

Causes and Clinical Features of 41 Patients with Hemophagocytic Syndrome

Table 2

Laboratory Findings Regarding 41 Patients with Hemophagocytic Syndrome

Table 3

Patients Characteristics Associated with Death of 41 Cases with Hemophagocytic Syndrome by Univariate Analysis

^aOdds ratio, ^bconfidence interval, ^cnot defined

Table 4

Patients Characteristics Associated with Death of 41 Cases of Hemophagocytic Syndrome Multivariate Analysis by Cox Regression Hazard Model

^anot defined

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ORCID iDs: Shin-Woo Kim
https://orcid.org/http://orcid.org/0000-0002-3755-8249
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