Acute kidney injury in adults with hemophagocytic lymphohistiocytosis

Background: Acute kidney injury (AKI) in the setting of hemophagocytic lymphohistiocytosis (HLH) is poorly characterized. This study aims to describe the incidence, clinical and biological features, and outcome associated with AKI in this population.

Study design: Case series.

Setting & participants: Patients with secondary HLH admitted to a single center from February 2007 through January 2013. 95 patients were included in the study.

Predictor: AKI.

Outcomes: Recovery of kidney function, 6-month mortality, and complete remission of the underlying disease.

Measurements: AKI was defined according to the KDIGO 2012 guideline. Recovery of kidney function was defined as improvement in serum creatinine level, with return to baseline serum creatinine level ±26.5μmol/L.

Results: HLH was related to hematologic malignancy in 73 (77%), infectious disease in 21 (22%), and autoimmune disease in 9 (10%) patients and was multifactorial in 10 (11%) patients. The cause was undetermined in 2 (2%) patients. The incidence of AKI during HLH is high (62%), and 59% of the AKI population required renal replacement therapy. Main causes of AKI were acute tubular necrosis (49%), hypoperfusion (46%), tumor lysis syndrome (29%), or HLH-associated glomerulopathies (17%). At 6 months, 32% of the patients with AKI had chronic kidney disease. Two factors were associated independently with 6-month mortality by multivariable analysis: AKI stage ≥ 2 (OR, 2.61; 95% CI, 1.08-6.29; P=0.03) and an underlying hematologic malignancy (OR, 3.1; 95% CI, 1.05-9.14; P=0.04). In patients with hematologic malignancy, AKI was associated with lower 6-month complete remission (non-AKI, 25%; AKI patients, 5%; P=0.05).

Limitations: Retrospective study, lack of histologic data.

Conclusions: AKI in patients with HLH is frequent and adversely affects remission and survival. Early intensive management, including administration of etoposide, nephrotoxic drug withdrawal, prevention of tumor lysis syndrome, or aggressive supportive care, might improve kidney function and survival.