Higher Fatality for Severe Fever with Thrombocytopenia Syndrome Complicated by Hemophagocytic Lymphohistiocytosis

In Young Jung, Kwangjin Ahn, Juwon Kim, Jun Yong Choi, Hyo Youl Kim, Young Uh, YoungKeun Kim

Research output: Contribution to journalArticle

Abstract

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious zoonosis caused by the SFTS virus. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome associated with excessive immune activation. Cytokine storms are often seen in both SFTS and HLH, resulting in rapid disease progression and poor prognosis. The aim of this study was to identify whether SFTS cases complicated by HLH are related to higher rates of mortality. Descriptive analysis of the frequency of clinical and laboratory data, complications, treatment outcomes, and HLH-2004 criteria was performed. Cases presenting with five or more clinical or laboratory findings corresponding to the HLH-2004 diagnostic criteria were defined as SFTS cases complicated by HLH. Eighteen cases of SFTS were identified during a 2-year study period, with a case-fatality proportion of 22.2% (4 among 18 cases, 95% confidence interval 9%-45.2%). SFTS cases complicated by HLH were identified in 33.3% (6 among 18 cases). A mortality rate of 75% (3 among 4 cases) was recorded among SFTS cases complicated by HLH. Although there were no statistically significant differences in outcomes, fatal cases exhibited more frequent correlation with HLH-2004 criteria than non-fatal cases [3/14 (21.4%) vs. 3/4 (75%), p=0.083]. In conclusion, the present study suggests the possibility that SFTS cases complicated by HLH are at higher risk of poor prognosis.

Original languageEnglish
Pages (from-to)592-596
Number of pages5
JournalYonsei medical journal
Volume60
Issue number6
DOIs
Publication statusPublished - 2019 Jun 1

Fingerprint

Hemophagocytic Lymphohistiocytosis
Thrombocytopenia
Fever
Fatal Outcome
Mortality
Zoonoses
Disease Progression
Confidence Intervals
Cytokines
Viruses

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

Jung, In Young ; Ahn, Kwangjin ; Kim, Juwon ; Choi, Jun Yong ; Kim, Hyo Youl ; Uh, Young ; Kim, YoungKeun. / Higher Fatality for Severe Fever with Thrombocytopenia Syndrome Complicated by Hemophagocytic Lymphohistiocytosis. In: Yonsei medical journal. 2019 ; Vol. 60, No. 6. pp. 592-596.
@article{686e95355dbe40838d83a672468e5d2c,
title = "Higher Fatality for Severe Fever with Thrombocytopenia Syndrome Complicated by Hemophagocytic Lymphohistiocytosis",
abstract = "Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious zoonosis caused by the SFTS virus. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome associated with excessive immune activation. Cytokine storms are often seen in both SFTS and HLH, resulting in rapid disease progression and poor prognosis. The aim of this study was to identify whether SFTS cases complicated by HLH are related to higher rates of mortality. Descriptive analysis of the frequency of clinical and laboratory data, complications, treatment outcomes, and HLH-2004 criteria was performed. Cases presenting with five or more clinical or laboratory findings corresponding to the HLH-2004 diagnostic criteria were defined as SFTS cases complicated by HLH. Eighteen cases of SFTS were identified during a 2-year study period, with a case-fatality proportion of 22.2{\%} (4 among 18 cases, 95{\%} confidence interval 9{\%}-45.2{\%}). SFTS cases complicated by HLH were identified in 33.3{\%} (6 among 18 cases). A mortality rate of 75{\%} (3 among 4 cases) was recorded among SFTS cases complicated by HLH. Although there were no statistically significant differences in outcomes, fatal cases exhibited more frequent correlation with HLH-2004 criteria than non-fatal cases [3/14 (21.4{\%}) vs. 3/4 (75{\%}), p=0.083]. In conclusion, the present study suggests the possibility that SFTS cases complicated by HLH are at higher risk of poor prognosis.",
author = "Jung, {In Young} and Kwangjin Ahn and Juwon Kim and Choi, {Jun Yong} and Kim, {Hyo Youl} and Young Uh and YoungKeun Kim",
year = "2019",
month = "6",
day = "1",
doi = "10.3349/ymj.2019.60.6.592",
language = "English",
volume = "60",
pages = "592--596",
journal = "Yonsei Medical Journal",
issn = "0513-5796",
publisher = "Yonsei University College of Medicine",
number = "6",

}

Higher Fatality for Severe Fever with Thrombocytopenia Syndrome Complicated by Hemophagocytic Lymphohistiocytosis. / Jung, In Young; Ahn, Kwangjin; Kim, Juwon; Choi, Jun Yong; Kim, Hyo Youl; Uh, Young; Kim, YoungKeun.

In: Yonsei medical journal, Vol. 60, No. 6, 01.06.2019, p. 592-596.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Higher Fatality for Severe Fever with Thrombocytopenia Syndrome Complicated by Hemophagocytic Lymphohistiocytosis

AU - Jung, In Young

AU - Ahn, Kwangjin

AU - Kim, Juwon

AU - Choi, Jun Yong

AU - Kim, Hyo Youl

AU - Uh, Young

AU - Kim, YoungKeun

PY - 2019/6/1

Y1 - 2019/6/1

N2 - Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious zoonosis caused by the SFTS virus. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome associated with excessive immune activation. Cytokine storms are often seen in both SFTS and HLH, resulting in rapid disease progression and poor prognosis. The aim of this study was to identify whether SFTS cases complicated by HLH are related to higher rates of mortality. Descriptive analysis of the frequency of clinical and laboratory data, complications, treatment outcomes, and HLH-2004 criteria was performed. Cases presenting with five or more clinical or laboratory findings corresponding to the HLH-2004 diagnostic criteria were defined as SFTS cases complicated by HLH. Eighteen cases of SFTS were identified during a 2-year study period, with a case-fatality proportion of 22.2% (4 among 18 cases, 95% confidence interval 9%-45.2%). SFTS cases complicated by HLH were identified in 33.3% (6 among 18 cases). A mortality rate of 75% (3 among 4 cases) was recorded among SFTS cases complicated by HLH. Although there were no statistically significant differences in outcomes, fatal cases exhibited more frequent correlation with HLH-2004 criteria than non-fatal cases [3/14 (21.4%) vs. 3/4 (75%), p=0.083]. In conclusion, the present study suggests the possibility that SFTS cases complicated by HLH are at higher risk of poor prognosis.

AB - Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious zoonosis caused by the SFTS virus. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome associated with excessive immune activation. Cytokine storms are often seen in both SFTS and HLH, resulting in rapid disease progression and poor prognosis. The aim of this study was to identify whether SFTS cases complicated by HLH are related to higher rates of mortality. Descriptive analysis of the frequency of clinical and laboratory data, complications, treatment outcomes, and HLH-2004 criteria was performed. Cases presenting with five or more clinical or laboratory findings corresponding to the HLH-2004 diagnostic criteria were defined as SFTS cases complicated by HLH. Eighteen cases of SFTS were identified during a 2-year study period, with a case-fatality proportion of 22.2% (4 among 18 cases, 95% confidence interval 9%-45.2%). SFTS cases complicated by HLH were identified in 33.3% (6 among 18 cases). A mortality rate of 75% (3 among 4 cases) was recorded among SFTS cases complicated by HLH. Although there were no statistically significant differences in outcomes, fatal cases exhibited more frequent correlation with HLH-2004 criteria than non-fatal cases [3/14 (21.4%) vs. 3/4 (75%), p=0.083]. In conclusion, the present study suggests the possibility that SFTS cases complicated by HLH are at higher risk of poor prognosis.

UR - http://www.scopus.com/inward/record.url?scp=85066818843&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85066818843&partnerID=8YFLogxK

U2 - 10.3349/ymj.2019.60.6.592

DO - 10.3349/ymj.2019.60.6.592

M3 - Article

VL - 60

SP - 592

EP - 596

JO - Yonsei Medical Journal

JF - Yonsei Medical Journal

SN - 0513-5796

IS - 6

ER -