Hepatobiliary Dysfunction and Disseminated Intravascular Coagulation Increase Risk of Mortality in Pediatric Hemophagocytic Lymphohistiocytosis.
Hepatobiliary Dysfunction and Disseminated Intravascular Coagulation Increase Risk of Mortality in Pediatric Hemophagocytic Lymphohistiocytosis.
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DOI：
10.1097/pcc.0000000000001684
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发表时间：
2018-10
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Hemophagocytic lymphohistiocytosis (HLH) poses significant challenges due to limited tools to guide clinical decisions in a population at high risk of death. We sought to assess whether disseminated intravascular coagulation (DIC) and hepatobiliary dysfunction (HBD), significant comorbidities seen in critical care settings, would identify HLH patients with increased risk of mortality. Retrospective chart review. Single-center pediatric intensive care unit All patients admitted to a tertiary care children’s hospital diagnosed with HLH from 2005 – 2012 None Forty-three patients were diagnosed with HLH with median age of 61 months. The 5-year overall survival was 51% (22/43). Univariate analyses revealed ferritin levels > 10,000 (ng/mL), international normalized ratio >1.5, or platelet counts < 100,000/mcL at initiation of dexamethasone were individually associated with mortality. Development of DIC, HBD or both increased the likelihood of death in HLH patients (relative risk; 95% confidence interval) (6; 1.4 – 34; p<0.05), (4.1; 1.8 – 10; p<0.05), and (7.5; 1.8 – 42; p<0.05). Of 12 autopsies performed, 75% had at least one active infection, 66% had chronic lymphopenia, 50% had lymphocyte depletion in the spleen, thymus or bone marrow, 42% had evidence of microvascular thrombosis, and 92% had evidence of hepatocellular injury. HLH continues to have high mortality with HLH-1994/2004 (dexamethasone/etoposide), the current standard of care for all children with HLH. HLH patients who developed DIC, HBD or both had higher risk of death with mortalities of 60, 77 and 77% respectively. Phenotypic classifications are urgently needed to guide individualized treatment strategies to improve outcomes for children with HLH.
