Risk factors for early fatal outcomes among children with hemophagocytic lymphohistiocytosis (HLH): a single-institution case-series in Vietnam

Type Journal Article - Pediatric Hematology and Oncology
Title Risk factors for early fatal outcomes among children with hemophagocytic lymphohistiocytosis (HLH): a single-institution case-series in Vietnam
Author(s)
Volume 31
Issue 3
Publication (Day/Month/Year) 2014
Page numbers 271-281
URL http://www.tandfonline.com/doi/abs/10.3109/08880018.2013.858198
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal hematological syndrome that
causes a disturbance of the immune system. Overall mortality of HLH is greater than 50% and
the majority of patients who die do so within the first 8 weeks of chemotherapy treatment. To
find clinical parameters relating to high-risk HLH patients, this study examined associations between
an early fatal outcome and potential prognostic clinical factors and laboratory findings on
admission. Eighty-nine pediatric HLH patients were prospectively recruited in Children’s Hospital
No. 1, Ho-Chi-Minh City, Vietnam, during the period from January 2010 to August 2012. Associations
between early fatal outcome and clinical and laboratory findings, including a cerebrospinal
fluid examination and virological test on admission, were examined. During the 8-week therapy,
25 (28%) HLH patients died. Persistent fever (>2 weeks), severe thrombocytopenia (<75 × 109/L),
hyperbilirubinemia, and prolonged activated partial thromboplastin time (APTT) (>33 sec) were
significant risk factors of early fatal outcome. Multivariate logistic regression analysis revealed that
thrombocytopenia and prolonged APTT (P for trend was 0.054 and 0.013, respectively) were independently
associated with the early fatal outcome. Persistent fever, severe thrombocytopenia,
hyperbilirubinemia, and prolonged APTT on admission will be useful and practical predictors to
determine high-risk HLH patients.

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