Iç Hastalıkları Yoğun Bakım Ünitesinde Tedavi Edilen Ağır Sepsis Hastalarında Hemofagositik Lenfohistiyositozun Araştırılması

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threating condition characterized with uncontrolled inflamation, prolonged fever, splenomegaly and cytopenia. HLH has common clinical and laboratory features with systemic inflammatory response syndrome (SIRS), sepsis and severe sepsis. Diagnostic criteria published by Histiocyte Society for HLH are consistent with sepsis. Therefore, it is thougth that pathogenesis of patients with sepsis may be underlying hemophagocytosis. In this study, our aim is to investigate patients for HLH who were treated with severe sepsis and had bicytopenia. Between June 2011-September 2012, 385 patients who were hospitalized in Hacettepe University, Faculty of Medicine, Adult Hospital Medical Intensive Care Unit were evaluated. In these process, 13 of those patients with severe sepsis and non-responsive to treatment and who developed at least bicytopenia were included in this study. Within the framework of diagnostic criteria for HLH patients, triglyceride, ferritin, fibrinogen levels were analyzed. During follow-up, bone marrow aspiration and biopsy specimens were obtained in patients with ongoing cytopenias. Peripheral blood samples were collected and stored for later evaluation for the two of the diagnostic criteria of HLH; Natural Killer (NK) activity and interleukin-2 receptor (IL-2R) levels. At the end of the study, all patients died. After the final evaluation, ten of 13 patients had at least five of the eight criteria according to criteria of the Histiocyte Society. Only one patient was diagnosed as HLH and received treatment during follow up. This study emphasizes to consider the possibility of HLH and need of rapid assessment in patients followed up with severe sepsis who were resistant to treatment with bicytopenia in intensive care. As case series, this study reveals that HLH might be more than expected and diagnosed.