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Clinical characteristics of liver failure with hemophagocytic lymphohistiocytosis
- Source :
- Scientific Reports, Scientific Reports, Vol 9, Iss 1, Pp 1-6 (2019)
- Publication Year :
- 2019
- Publisher :
- Springer Science and Business Media LLC, 2019.
-
Abstract
- Liver failure with hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome with high mortality. The aim of this study was to decipher clinical and laboratory characteristics of hemophagocytic lymphohistiocytosis after definite diagnosis of liver failure and to provide clues for early diagnosis and treatment of HLH in patients with liver failure. Eleven patients diagnosed with liver failure and HLH were retrospectively investigated in this study. All patients presented with jaundice, persistent high-grade fever, pancytopenia, splenomegaly, evidence of hemophagocytes in the bone marrow and laboratory abnormalities indicating HLH. The average interval from the earliest diagnosis of liver failure to a definitive diagnosis of HLH was 17.27 days. Six (54.55%) patients died during follow-up. For patients with liver failure after admission and subsequently definitively diagnosed with HLH, bilirubin and INR were significantly decreased. HLH is definitely diagnosed at an intermediate or late stage when patients have already suffered from liver failure. The initial dose of glucocorticoid (methylprednisolone) was decreased to 1–1.5 mg/kg/d and gradually reduced thereafter. In conclusion, for patients with liver failure, HLH should be screened as early as possible upon persistent fever, splenomegaly and unexplained pancytopenia. For patients with liver failure and HLH, the dosage of glucocorticoid should be reduced to avoid serious side effects.
- Subjects :
- Male
0301 basic medicine
Pancytopenia
lcsh:Medicine
Gastroenterology
Hepatitis
chemistry.chemical_compound
0302 clinical medicine
hemic and lymphatic diseases
Medicine
lcsh:Science
Multidisciplinary
Middle Aged
Jaundice
musculoskeletal system
medicine.anatomical_structure
Methylprednisolone
Female
medicine.symptom
hormones, hormone substitutes, and hormone antagonists
Glucocorticoid
medicine.drug
Adult
endocrine system
medicine.medical_specialty
Adolescent
Fever
Bilirubin
Article
Lymphohistiocytosis, Hemophagocytic
Young Adult
03 medical and health sciences
Internal medicine
Humans
International Normalized Ratio
Liver diseases
Aged
Retrospective Studies
Hemophagocytic lymphohistiocytosis
business.industry
lcsh:R
fungi
Retrospective cohort study
medicine.disease
030104 developmental biology
chemistry
Splenomegaly
lcsh:Q
Bone marrow
business
Liver Failure
030217 neurology & neurosurgery
Follow-Up Studies
Subjects
Details
- ISSN :
- 20452322
- Volume :
- 9
- Database :
- OpenAIRE
- Journal :
- Scientific Reports
- Accession number :
- edsair.doi.dedup.....d7de16d66d754360413d98eea0681d37