The role of 18 F-FDG PET/CT in the management of patients with secondary haemophagocytic lymphohistiocytosis.

AIM: To investigate the ability of combined 2-[18 F]-fluoro-2-deoxy-d-glucose (18 F-FDG) positron-emission tomography (PET)/computed tomography (CT) to determine potential causes of secondary haemophagocytic lymphohistiocytosis (sHLH) and to predict prognosis.

MATERIAL AND METHODS: Forty-three patients (male/female 20/23, median age 48.5 years), who were diagnosed with sHLH and underwent FDG-PET/CT before treatment, were retrospectively reviewed. The clinical characteristics were compared to identify the predictors of high-yield FDG-PET/CT. Univariate and multivariate analyses were conducted to identify factors associated with survival. Statistical analysis was performed using SPSS version 19.0.

RESULTS: PET results were helpful in 65.1% (28/43), whilst non-contributory in 34.9% (15/43) of patients with regard to the final diagnosis. Lymphoma was the most common (25/43) reason for sHLH, and patients with focal FDG uptake were more likely to be diagnosed with underlying diseases. C-reactive protein (CRP) was found to be a good indicator for the usefulness of PET/CT in HLH patients. Multivariate analysis showed that therapy regimen (hazard ratio [HR]=4.99, p=0.026), fibrinogen (FBG) <1.5 g/l (HR=3.87, p=0.049) and spleen:mediastinum ratio (SP/M) (HR=7.44, p=0.006) were independent prognostic factors for survival.

CONCLUSION: FDG-PET/CT could be a useful technique for detecting underlying diseases causing sHLH. CRP was a useful predictor of FDG-PET/CT effectiveness. Therapy regimen, FBG level, and SP/M were independent prognostic factors for HLH survival.