Characteristics and outcomes of Hemophagocytic Lymphohistiocytosis In children

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition associated with excessive or uncontrolled release of proinflammatory cytokines. Clinical manifestations include fever, splenomegaly, high serum ferritin level and cytopenia. Early diagnosis and proper management would effectively reduce mortality of this lethal condition.
Objectives: To study the etiologies, clinical characteristics, outcomes of treatment in children with HLH and determine prognostic factors for early outcomes and causes of death in children with HLH.                                                                                                                Methods: In this retrospective descriptive study, the medical records of HLH children, who were treated at Maharat Nakhon Ratchasima Hospital from 1 January 2008 to 30 June 2018 have been reviewed.                                                                                                    Results: Of the 48 children diagnosed with HLH, nineteen patients (40%) with infectious-associated hemophagocytic syndrome (IAHS), three patients (6%) with malignancy associated hemophagocytic syndrome, and two patients (4%) with macrophage activation syndrome. Causes of early death within 7 days included infection and multiorgan failure, which accounted for 8% of total mortality rate 29%. In comparison between the survivors and deaths, thrombocytopenia (<50,000/mm3), elevated total or
direct bilirubin (>2 mg/dL), and hypofibrinogenemia (<100 mg/dL) were statistically significant found in mortality group (P-value = 0.0005, 0.0088, 0.01, 0.015, respectively). However, age, ferritin level and neutrophil count were not statistically significant difference.
Conclusions: This study affirmed that HLH is a life-threatening condition with high mortality. High serum total and direct bilirubin, thrombocytopenia and hypofibrinogenemia conferred adverse prognostic outcomes in those HLH children.