Abstract
Purpose The significance of positron emission tomography/computed tomography (PET–CT) in identifying patients with lymphoma-associated hemophagocytic lymphohistiocytosis (LAHLH) when pathological evidence is unavailable remains uncertain.MethodsIn this retrospective study, 44 HLH patients who underwent PET–CT before clinical treatment were enrolled, and 18 of them were highly suspected as LAHLH by PET–CT. We compared the PET–CT parameters between confirmed LAHLH and non-LAHLH patients. The efficacy of initial therapies for highly suspected LAHLH patients was analyzed as well.Results We found that the SUVSp, SUVBM, SUVLN, SUVmax, SUVLN/Li, and SUVmax/Li in LAHLH group were significantly higher than those in non-LAHLH group (p = 0.003, p = 0.034, p = 0.003, p < 0.001, p = 0.039, and p = 0.035, respectively). HLH patients with an SUVmax value >5.5, an SUVLN value >3.3, and an SUVSp value >4.8 were more likely to be LAHLH (p < 0.001, p = 0.003, and p = 0.003, respectively). And the incidence of multiple lymphadenopathy with increased FDG uptake or the incidence of multiple bone lesions in LAHLH patients was significantly higher than those in non-LAHLH group (92.9 vs. 35.7 %, p = 0.004; 42.9 vs. 0 %, p = 0.016, respectively). Furthermore, by comparing the efficacy of initial therapies for highly suspected LAHLH patients (n = 18), we indicated that the CR rate was significantly higher in lymphoma-chemotherapy group than in immunosuppressive therapy group (90 and 25 %, respectively; p = 0.013). OS analysis revealed that highly suspected LAHLH patients treated with lymphoma-chemotherapy had better prognosis (264 days) than those treated with immunosuppressive therapy (15 days) (p < 0.0001).ConclusionsWhen pathological evidence is absent, PET–CT may play an important role in identifying HLH patients underlying lymphoma. Once highly suspected as LAHLH by PET–CT, lymphoma-chemotherapies that directly treat the underling lymphoma may have a relatively favorable effect and better clinical outcomes than immunosuppressive therapy.
Highlights
Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening syndrome of hyper-inflammation, caused by uncontrolled activation and proliferation of lymphocytes and antigen-presenting cells (Filipovich 2009)
We summarized the positron emission tomography/computed tomography (PET–CT) characteristics of the 44 patients with Secondary HLH (sHLH) who were admitted into our hospital and underwent the PET–CT examination
To better distinguish lymphoma-associated HLH (LAHLH) patients from HLH patients, patients were divided into three groups: LAHLH group (n = 14), non-LAHLH group (n = 14), and unexplained causes group (n = 16)
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening syndrome of hyper-inflammation, caused by uncontrolled activation and proliferation of lymphocytes and antigen-presenting cells (macrophages, histiocytes) (Filipovich 2009). The rapid progression and extremely high mortality rate of HLH despite proper management (Kaito et al 1997) frustrate both patients and physicians. Primary HLH, known as familial HLH (FHL), is a disease caused by mutations in the genes with a median survival of less than 2 months after diagnosis if not treated (Henter et al 2007). Secondary HLH (sHLH) may develop as a result of strong immune activation in association with severe infections, malignancies, or autoimmune diseases. It is, respectively, classified as infection-associated HLH (IAHLH), malignancyassociated HLH (MAHLH) (Janka et al 1998), and rheumatic autoimmune diseases-associated HLH (RAHLH). Further studies about identifying the causative agents and finding efficacious regimens to these specific sHLH patients are seldom reported
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