Abstract

Background and Aims

Hemophagocytic lymphohistiocytosis is a clinical syndrome caused by a cytokine storm and phagocytosis of blood cells. Acute kidney injury is typically associated with a poor prognosis in hemophagocytic lymphohistiocytosis. In the present study, a retrospective analysis of patient data was performed to identify risk factors associated with acute kidney injury in hemophagocytic lymphohistiocytosis.

Methods

All patients included  in the study were diagnosed with hemophagocytic lymphohistiocytosis between January 2009 and July 2019. Acute kidney injury was diagnosed according to the Kidney Disease Improving Global Outcomes guidelines, according to the 2012 update. We collected the general information of the patients, clinical manifestations, treatments, as well as laboratory data from the electronic medical records.

Results

We analyzed 600 patients with hemophagocytic lymphohistiocytosis. Serum phosphorus, need for vasopressors, heart failure, gastrointestinal symptoms, disseminated intravascular coagulation, high heart rate at admission, total bilirubin and albumin levels were independently associated with an increased risk of developing acute kidney injury. Independent risk factors for in-hospital mortality were administration of vasopressors, acute kidney injury stage III, baseline Cystatin-C, total bilirubin, number of days of glucocorticoid therapy, fibrinogen level and presence of multi-organ failure.

Conclusion

Patients with hemophagocytic lymphohistiocytosis usually exhibit high hospital mortality, particularly in the presence of acute kidney injury. The risk factors for the occurrence of acute kidney injury and increased mortality identified in the study may assist clinicians in the prevention of acute kidney injury, and in its timely treatment in patients affected by hemophagocytic lymphohistiocytosis, to ultimately improve prognosis.

Graphic abstract