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Clinical features and prognostic factors of adult secondary hemophagocytic syndrome: Analysis of 47 cases.

Abstract This study aimed to investigate the relationship between clinical features and prognosis of adult secondary hemophagocytic syndrome (HPS).A retrospective analysis was conducted on the pathogenesis, clinical manifestations, laboratory examinations, treatment options, and prognosis of 47 patients with adult secondary HPS diagnosed from January 2013 to December 2015.The average age at disease onset was (46.26 ± 18.98) years with a male:female ratio of 1:1.14. Thirteen patients died, with the highest mortality rate in patients with HPS underlying blood system malignancy (33.33%, 2/6). The mortality rate in patients with HPS underlying autoimmune disorders was the lowest (18.75%, 3/16). The Kaplan-Meier analysis indicated that signs of hemorrhage, pulmonary and nervous system involvement, serous effusion, and decrease in the blood platelet count were associated with death. The Cox regression analysis revealed that signs of hemorrhage, pulmonary involvement, serous effusion, and nervous system involvement were independent risk factors of patient death.Adult secondary HPS has multiple etiologies and diversified clinical features. The risk of death increases in patients with signs of hemorrhage, serous effusion, pulmonary involvement, and nervous system involvement.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 28562543
OWN - NLM
STAT- MEDLINE
DA  - 20170531
DCOM- 20170626
LR  - 20170626
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 22
DP  - 2017 Jun
TI  - Clinical features and prognostic factors of adult secondary hemophagocytic
      syndrome: Analysis of 47 cases.
PG  - e6935
LID - 10.1097/MD.0000000000006935 [doi]
AB  - This study aimed to investigate the relationship between clinical features and
      prognosis of adult secondary hemophagocytic syndrome (HPS).A retrospective
      analysis was conducted on the pathogenesis, clinical manifestations, laboratory
      examinations, treatment options, and prognosis of 47 patients with adult
      secondary HPS diagnosed from January 2013 to December 2015.The average age at
      disease onset was (46.26 +/- 18.98) years with a male:female ratio of 1:1.14.
      Thirteen patients died, with the highest mortality rate in patients with HPS
      underlying blood system malignancy (33.33%, 2/6). The mortality rate in patients 
      with HPS underlying autoimmune disorders was the lowest (18.75%, 3/16). The
      Kaplan-Meier analysis indicated that signs of hemorrhage, pulmonary and nervous
      system involvement, serous effusion, and decrease in the blood platelet count
      were associated with death. The Cox regression analysis revealed that signs of
      hemorrhage, pulmonary involvement, serous effusion, and nervous system
      involvement were independent risk factors of patient death.Adult secondary HPS
      has multiple etiologies and diversified clinical features. The risk of death
      increases in patients with signs of hemorrhage, serous effusion, pulmonary
      involvement, and nervous system involvement.
FAU - Guo, Yiqun
AU  - Guo Y
AD  - aDepartment of Infectious Diseases and Clinical Microbiology bDepartment of
      Internal medicine, Beijing Chao-yang Hospital, Capital Medical University,
      Beijing, China.
FAU - Bai, Yu
AU  - Bai Y
FAU - Gu, Li
AU  - Gu L
LA  - eng
PT  - Journal Article
PT  - Observational Study
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Biomarkers)
SB  - AIM
SB  - IM
MH  - Adult
MH  - Age of Onset
MH  - Biomarkers/blood
MH  - Female
MH  - Humans
MH  - Kaplan-Meier Estimate
MH  - Lymphohistiocytosis,
      Hemophagocytic/*diagnosis/etiology/mortality/*physiopathology
MH  - Male
MH  - Middle Aged
MH  - Platelet Count
MH  - Prognosis
MH  - Proportional Hazards Models
MH  - Retrospective Studies
PMC - PMC5459708
EDAT- 2017/06/01 06:00
MHDA- 2017/06/27 06:00
CRDT- 2017/06/01 06:00
AID - 10.1097/MD.0000000000006935 [doi]
AID - 00005792-201706020-00019 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Jun;96(22):e6935. doi: 10.1097/MD.0000000000006935.